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Biomedical subjects

P Vajro

Publications and source records attributed to P Vajro.

At least 37 records · Page 2Linked to original sources
PubMed1999

Low virological response to interferon in children with chronic hepatitis C.

BACKGROUND/AIMS: Children with chronic hepatitis C were recently found to have higher rates of sustained response to interferon compared to adults. The aim of this study was to verify the response to interferon using frequent viremia measurements. METHODS: Sera from 25 children (13 males; mean age 7.9 years) with chronic hepatitis C, treated with recombinant alpha-2b interferon for 12 months, were tested for liver function tests and viremia levels for a median of 27.5 months. Autoantibodies were evaluated during and after interferon. RESULTS: Fifteen patients completed 12 months of interferon; treatment was stopped in 10 other patients. In 11 (44%) patients viremia was undetectable already at the second administration of interferon; one of them remained viremia-free up to the end of follow-up and had persistently normal alanine-aminotransferase levels (complete sustained responder). A complete sustained response was observed only in one other patient, who normalized alanine aminotransferase and cleared viremia from the 3rd month of therapy. Three patients with persistent viremia normalized alanine-aminotransferase from the 3rd week of therapy up to the end of follow-up (biochemical sustained responders). Viremia was undetectable during treatment in four patients, who stopped interferon because of worsening in hypertransaminasemia. Three of these four patients were anti-liver-kidney microsomal type 1-positive. CONCLUSIONS: In this study the response rate to interferon was very low and viremia and transaminase findings were often discordant.

Adolescent↗
PubMed1999

Chronic cryptogenic hepatitis in childhood is unrelated to hepatitis G virus.

OBJECTIVES: The aim of this study was to define the features of chronic cryptogenic hepatitis (CCH) in childhood and to investigate whether it is related to hepatitis G virus infection. METHODS: Forty-six children (24 males; age range, 1.5 to 17 years) with CCH were studied. CCH was diagnosed when serum alanine aminotransferase concentrations were more than 1.5 times normal for longer than 6 months without any apparent cause of liver disease. RESULTS: No patient had acute symptomatic onset or had received a blood transfusion. Three had undergone minor surgical procedures. All appeared to be healthy during follow-up (median, 4.2 years; range, 1 to 10 years). Hypertransaminasemia was the only aberrant liver function test. Elevated serum alanine aminotransferase values alternated with normal values in 40 children (86.9%). Five children (10.8%) had a spontaneous sustained (>12 months) remission of hypertransaminasemia. Twelve (26%) had laboratory signs of autoimmunity, but none fulfilled the criteria for autoimmune hepatitis. Of 20 children who underwent liver biopsy, 13 (65%) had minimal chronic hepatitis, 4 (20%) had mild chronic hepatitis and 3 (15%) had moderate chronic hepatitis. Serum hepatitis G virus RNA was detected in 2 girls (4%) whose risk factor was a hepatitis G virus-infected mother and a minor surgical procedure, respectively. In 12 families at least 1 other member had chronic liver disease. CONCLUSIONS: Childhood CCH seems to be a symptomless disease characterized by isolated hypertransaminasemia with onset during the first 4 years of life and mild to moderate histologic liver lesions. Although the frequency of spontaneous remissions is low, childhood CCH seems, in the short run, to be a nonprogressive disease. Hepatitis G virus does not play a major role in CCH.

Adolescent↗
PubMed1998

Macroenzyme investigation and monitoring in children with persistent increase of aspartate aminotransferase of unexplained origin.

Ten children with asymptomatic persistent cryptogenic increased serum levels of aspartate aminotransferase (AST) were screened for detection and monitoring of AST macroenzyme (macroAST). MacroAST was found in 4 patients; their serum AST levels were significantly higher than in those without biochemical evidence of macroAST (mean +/- SD: 515 +/- 433 and 78 +/- 16 IU/L, respectively; P = .0095). MacroAST was a persistent, benign phenomenon and was probably not congenital.

Aspartate Aminotransferases↗
PubMed1997

Penicillamine-related neurologic syndrome in a child affected by Wilson disease with hepatic presentation.

OBJECTIVES: To describe a case of penicillamine-related neurologic symptoms in a 9-year-old boy affected by asymptomatic Wilson disease with hepatic presentation; to compare this case with similar cases in adults; and to discuss the role of zinc therapy as an alternative treatment for patients who have an adverse reaction to penicillamine therapy. SETTING: Referral hospital. MAIN OUTCOME MEASURE: The occurrence of a neurologic syndrome that severely impaired a child's usual daily activities and his health-related quality of life after the institution of penicillamine therapy. RESULTS: Initial penicillamine therapy was chronologically related to the development of progressive neurologic deterioration in the absence of other causes of neurologic syndrome. The discontinuation of penicillamine therapy and the initiation of zinc therapy were followed by a prompt disappearance of neurologic symptoms and a return to neurologic baseline status. CONCLUSIONS: Penicillamine therapy, even in children affected by Wilson disease with hepatic presentation alone and without neurologic disease at the beginning of treatment, may trigger neurologic symptoms. Zinc therapy may be a satisfactory alternative.

Child↗
PubMed1997

Hepatitis G and C virus coinfection in children.

Infection with the hepatitis G virus (HGV), as indicated by the presence of HGV ribonucleic acid, was sought in 57 children with chronic hepatitis C virus infection. HGV infection was found in 2 children (3.5%), or 14% of the babies whose mothers were former drug abusers. Maternal drug abuse is an important risk factor for hepatitis G and C virus coinfection in children in our area.

Adolescent↗
PubMed1997

Liver involvement in obese children. Ultrasonography and liver enzyme levels at diagnosis and during follow-up in an Italian population.

Our aim was to evaluate incidence and risk factors of liver involvement in obese Italian children as assessed by both ultrasonographic and biochemical parameters. In seventy-five consecutive obese children (age 9.5 +/- 2.9 years, males/females 41/34), serum levels of enzymes and ultrasonography of the liver were evaluated. Tests were repeated one, three, and six months after starting a moderate hypocaloric diet and an exercise program. Three obese children who were found to have chronic viral hepatitis were excluded from the study. Thirty-eight of 72 (53%) obese children had an ultrasonographic image of bright liver consistent with liver steatosis. The latter was severe in nine children, moderate in 16, and mild in 13. Eighteen obese children (25%) had elevated transaminase levels. Bright liver and hypertransaminasemia were not due to any of the most common causes of liver disease. Both were rapidly responsive to loss of weight, confirming that liver involvement was secondary to obesity and that steatosis or steatohepatitis rather than fibrosis were involved. Obesity duration not more than three years (odds ratio = 4.77), a higher degree of obesity (odds ratio = 2.09), and hypertransaminasemia (odds ratio = 2.15) appeared as important predictive factors of liver involvement at ultrasonography. Incidence of liver involvement assessed by means of ultrasonography is significantly higher than that revealed by measurement of serum liver enzymes. A short duration of obesity emerged as a potentially new risk factor of liver involvement in the pediatric obese population and needs to be confirmed in future studies.

Child↗
PubMed1997

Fas/Apo1 mutations and autoimmune lymphoproliferative syndrome in a patient with type 2 autoimmune hepatitis.

Inherited mutations of the Fas/Apo1/CD95 gene, a cell-surface receptor involved in cell death signaling and in the control of self-reactivity, characterize the recently identified autoimmune lymphoproliferative syndromes. A patient with type 2 autoimmune hepatitis with the immunologic and genetic features of autoimmune lymphoproliferative syndrome is described. The clinical picture was dominated by liver disease with hepatosplenomegaly and positivity for anti-liver-kidney microsome 1 and anti-liver-cytosol 1 antibodies. A marked increase in CD3+CD4-CD8-T lymphocytes and inherited mutations in Fas alleles that led to the expression of a soluble form of the protein were also found. Fas-mediated apoptosis was deficient in the patient as it was in her mother and her sister, who carried the same allele 2 mutation. This observation links type 2 autoimmune hepatitis, an organ-specific disease, with a genetically determined defect in peripheral tolerance control.

Alleles↗
PubMed1997

Side effects of alpha-interferon therapy and impact on health-related quality of life in children with chronic viral hepatitis.

BACKGROUND: Interferon (IFN) is standard therapy for chronic viral hepatitis in children. The aim of this study was to evaluate the side effects of alpha-interferon (IFN) in 94 consecutive children (58 males; age range, 3 to 14 years) affected by chronic viral hepatitis treated with different schedules ranging from 3 to 10 MU and from 3 to 12 months, and the impact of this therapy on health-related quality of life. METHODS: Side effects were evaluated with clinical and laboratory examinations and were recorded on a diary card. The health-related quality of life was evaluated with a modified version of the Sickness Impact Profile. RESULTS: All patients experienced at least one adverse reaction to IFN treatment; 80% had more than five side effects. There were no life-threatening reactions. Three children experienced severe reactions (febrile seizure, severe hypertransaminasemia and relapsing episodes of epistaxis, respectively) that required permanent IFN withdrawal. Another child had a febrile seizure requiring temporary IFN withdrawal. In seven children the neutrophil count fell below 1000/mm3 and promptly increased when IFN was temporarily discontinued. The remaining children had mild or moderate clinical and/or laboratory adverse reactions. Age, sex, viral etiology of chronic hepatitis and response to therapy were not significantly associated with the appearance of side effects. The pre-IFN health-related quality of life was good in all children; it deteriorated significantly during IFN therapy and returned to basal standards within 3 months after IFN withdrawal. No patient required suspension of IFN therapy because of worsening of health-related quality of life. CONCLUSION: Children have a low risk of developing severe IFN-induced side effects. Adverse reactions and worsening of health-related quality of life were tolerable and did not seem to be a limiting factor for IFN therapy in young candidates.

Adolescent↗
PubMed1997

Laparoscopy-guided biopsy in diagnosis of liver disorders in children.

To determine the safety and advantages of laparoscopic liver biopsy in pediatric liver disorders, we reviewed the medical records of 80 children affected by liver disease of various etiologies who underwent this procedure from 1986 to 1996. The main indicators for laparoscopic biopsy were increased risk of bleeding (i.e., mild to moderate coagulation abnormalities in patients probably affected by cirrhosis) and/or previous poorly informative blind needle liver biopsy (65 cases), and the need for a large amount of liver tissue for biochemical assays (10 cases). After inspection of the liver surface, at least two core biopsies were performed using a Tru-cut needle. We encountered difficulties with the biopsy in only four cases, due to a hard consistency of the liver. Bleeding time from the liver orifice was greatly reduced by positioning a fibrin plug (50-120 s vs 5-10 s, on average). In 15 patients, a large excisional biopsy was also successfully performed. Our results confirm an important role for laparoscopy in the diagnosis of cirrhosis (30% of bioptic false negative diagnoses in this series) and show that in selected cases laparoscopy-guided needle or excisional biopsy is an easy, useful and safe alternative to percutaneous blind liver biopsy.

Adolescent↗
PubMed1997

Cholestasis in newborn infants with perinatal asphyxia.

OBJECTIVE: The aim of this study was to characterize the liver involvement in infants who have both neonatal asphyxia and neonatal cholestasis. METHODS: We describe four asphyctic newborn infants (three born at term) who developed early (age 3.8 +/- 2.1 days) intrahepatic neonatal cholestasis and in whom tests for causes of neonatal liver damage were negative. RESULTS: The clinical picture and course were benign and similar to that of sporadic "idiopathic" neonatal hepatitis. Clinical signs and abnormal liver function tests tended to normalize within the first year of life in all. Conjugated bilirubin became normal at 6 months after the onset of cholestasis, while liver enzymes tended towards normal values thereafter, within 1 year of follow-up. Liver biopsy (taken in one patient) showed a typical picture of giant cell hepatitis; ultrastructure was nonspecific. SIGNIFICANCE: Our results suggest that isolated asphyxia should be taken into account as a potential causal factor in term or pre-term asphyctic newborns who develop early "idiopathic" neonatal cholestasis.

Asphyxia Neonatorum↗
PubMed1997

Percutaneous blind needle biopsy versus combined laparoscopic excisional and guided needle biopsy in the diagnosis of liver disorders in pediatric patients.

AIMS: The authors retrospectively analyzed the results of their experience with combined laparoscopic excisional and guided needle biopsy in the determination of chronic liver disease in children. PATIENTS AND METHODS: Between January 1986 and January 1996, at the Division of Pediatric Surgery of the "Federico II" University of Naples, eighty patients underwent laparoscopic liver biopsy to evaluate a chronic liver disease. Patient ages ranged between 50 days and 16 years (mean 5.8 years). There were 41 girls and 39 boys. Fifteen of these children (18.7%) were addressed to a surgeon because the previously performed percutaneous blind needle biopsy was not sufficiently informative or because the specimens were too small to allow chemical or biochemical tissue studies. In these 15 patients, we performed a combination of laparoscopic guided needle biopsy using a 14-gauge tru-cut needle together with an excisional biopsy on the right lobe, using two additional 5-mm trocars. RESULTS AND CONCLUSION: The post-operative course was uneventful for all the patients. All patients were discharged from hospital after 24 hours. A precise histological diagnosis was made and biochemical tissue studies were possible after laparoscopic biopsy in all 15 children. In our opinion, a combination of laparoscopic-guided needle biopsy and laparoscopic excisional biopsy is more reliable than simple biopsy alone for the diagnosis of cirrhosis in children with chronic liver disease.

Adolescent↗
PubMed1996

COACH syndrome: report of two brothers with congenital hepatic fibrosis, cerebellar vermis hypoplasia, oligophrenia, ataxia, and mental retardation.

Congenital hepatic fibrosis (CHF) is probably the most common cause of non-icteric hepatosplenomegaly and is encountered mainly in children and young adults. We describe here two brothers from healthy, non-consanguineous parents. The patients showed early hepatosplenomegaly, portal hypertension, and no apparent kidney involvement. Clinical and laboratory findings were similar in both patients. Liver biopsies showed the presence of broad septa of fibrous tissue containing abundant bile ducts, portal tracts enlarged by fibrosis, and preserved lobular architecture. The histological findings were suggestive of CHF. Ophthalmological assessment demonstrated visual impairment with mild exotropia, nystagmus, and oculomotor apraxia. Neurological examination showed moderate mental retardation and cerebellar ataxia. Brain MRI confirmed cerebellar malformation with inferior vermis hypoplasia. This pattern of defects is consistent with COACH syndrome (Cerebellar vermis hypoplasia, Oligophrenia, congenital Ataxia, Coloboma, Hepatic fibrocirrhosis) which has previously been reported in five other cases. Our report may contribute to a better delineation of the COACH syndrome phenotype in the spectrum of oculo-encephalohepato-renal disorders.

Abnormalities, Multiple↗
PubMed1996

Wilson disease mutations associated with uncommon haplotypes in Mediterranean patients.

This study reports 12 novel mutations of the Wilson disease (WD) gene which have been detected by the molecular analysis of 29 patients of Mediterranean descent carrying uncommon chromosomal haplotypes at the WD locus. These mutations include two nonsense, one splice site and nine missense. The missense mutations lie in regions of the WD gene critical for its function, such as the transmembrane region, the transduction domain and the ATP loop and ATP-binding domain, indicating that they are disease-causing mutations. These new findings improve our knowledge for the role played by functional domains on the ATP7B function.

Albania↗
PubMed1996

Non-organ specific autoantibodies in children with chronic hepatitis C.

BACKGROUND/AIMS: Recent studies in adult patients have established a relationship between hepatitis C virus infection and the presence of liver-kidney microsomal autoantibody type 1 (LKM1). Conversely, little is known regarding the relationship between hepatitis C and autoimmunity in children. In this study, we investigated non-organ specific autoantibodies in 40 otherwise healthy Italian children with chronic hepatitis C. METHODS: All but four patients included in the study were asymptomatic. Liver histology, obtained in 35, showed features ranging from minimal to mild chronic hepatitis. Autoantibodies were investigated by indirect immunofluorescence. HCV RNA was assayed by the polymerase chain reaction in 34 cases and viral genotypes were determined. RESULTS: Antinuclear antibodies were detected in three (7.5%) cases, one with a homogeneous pattern; smooth muscle autoantibodies in seven (17.5%) cases, always with V (vessels only) specificity and LKM1 in four (10%), at titers ranging from 1:20 and 1:2560. Clinical and virologic features did not significantly differ between autoantibody positive and negative cases, although infections with HCV genotypes 1a and 2 were more frequent in LKM1-positive patients. During observation, the child with the highest LKM1 titre was unsuccessfully treated with alpha interferon but responded to steroids. Twelve LKM1 negative children were also treated with interferon and one developed low LKM1 titers concomitant with an alanine aminotransferase flare. The sera of the five LKM1-positive children with investigated by immunoblotting with a human microsomal fraction and peptide 257-269 of cytochrome P450IID6. Only the serum of the child with the highest LKM1 titers was reactive. CONCLUSIONS: These results show that a consistent proportion of children with chronic hepatitis C circulate non-organ specific autoantibodies. The prevalence of LKM1 is greater than in adults and this could raise problems for the treatment of the disease with interferon. The analysis of LKM1 target antigens might help to identify putative cases of "true" autoimmune hepatitis with concomitant HCV infection that could benefit from steroid treatment.

Adolescent↗