Primary sclerosing cholangitis preceding Crohn's disease in a child with Down's syndrome.
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Child↗
Biomedical subjects
R Iorio
Publications and source records attributed to R Iorio.
Autoantibody prevalence in children with liver disease due to chronic hepatitis C virus (HCV) infection.
HCV infection and interferon-alpha (IFN-alpha) therapy have been associated with autoimmunity. To assess whether chronic liver disease (CLD) due to HCV infection or its treatment with IFN-alpha cause autoimmune manifestations, the prevalence of tissue autoantibodies in 51 children with chronic HCV infection and 84 with other CLD was analysed by standard techniques. Sixty-five percent of patients with chronic HCV infection, 66% with chronic hepatitis B infection and 60% with Wilson's disease were positive for at least one autoantibody. In the 51 subjects with chronic HCV infection (29 treated with IFN-alpha, 22 untreated), tested on 165 occasions over a median of 9 months (range 5-42 months), autoantibodies to nuclei (ANA), smooth muscle (SMA), gastric parietal cell (GPC) and/or liver kidney microsomal type 1 (LKM-1) were similarly prevalent in treated and untreated patients (90% versus 68%, P = 0.12). Positivity for SMA was present in 67%, GPC in 32%, ANA in 10%, LKM-1 in 8% of cases. Treatment with IFN-alpha had to be suspended due to transaminase elevation in one SMA-positive, one ANA-positive but in three of four LKM-1-positive patients. Our results show that: (i) autoantibodies are common in viral-induced hepatitis and Wilson's disease; (ii) positivity for SMA, GPC, ANA is part of the natural course of chronic HCV infection, their prevalence being unaffected by IFN-alpha; and (iii) IFN-alpha should be used cautiously in the treatment of LKM-1/HCV-positive patients.
Adolescent↗
Medial reconstruction during total knee arthroplasty for severe valgus deformity.
A painful arthritic knee with severe valgus deformity may be treated successfully with total knee arthroplasty using several techniques: constrained implant with lateral release, nonconstrained implant with lateral release and a thick tibial insert, or nonconstrained implant with lateral release and medial reconstruction. Eight patients with Type II valgus deformity were treated with nonconstrained total knee arthroplasty implants, lateral ITB release at the level of the tibial osteotomy, and proximal medial collateral ligament advancement with bone plug recession. The reconstruction led to predictably successful outcomes in all patients at 4- to 9-years followup. All patients were satisfied with the operation. All knees were stable with a functional range of motion at the time of last followup.
Aged↗
Chronic unexplained liver disease in children with primary immunodeficiency syndromes.
Liver disease may be found in patients with primary immunodeficiency syndromes because of the high risk of infection with hepatotropic viruses related to the treatment with blood derivatives. The prevalence of liver disease in these patients and its etiology, however, is still not completely understood. We have evaluated the prevalence and the etiology of liver disease in children with different forms of primary immunodeficiencies. Thirty patients included in the study underwent molecular studies to detect common hepatotropic viruses, including hepatitis C and G viruses. Liver involvement was found in 11 of 30 (36.6%) patients. All patients with liver disease had deficiencies of specific immunity, with a prevalence in this subgroup of 47.8%. Liver disease was more severe in patients with T and B cell combined immune disorders than in those with a selective T cell immunodeficiency. Moreover, the severity of the disease correlated with an overall more rapid fatal outcome. A viral etiology was found in only six of these patients, whereas in the remaining five patients, no cause of liver injury was identified. In the virally infected patients, hepatitis C virus was the most common viral agent. In patients with immunodeficiencies, there is a high prevalence of liver disease not fully explained on the basis of the common viral infections.
Adolescent↗
Muscular herniation of the lower extremities.
Fascial defects are encountered infrequently by physicians who manage disorders of the lower extremities. The correct diagnosis of this condition is important, because the treatment is forthright and will bring the patient relief from symptoms. This article reviews the literature and attempts to aid the clinician in understanding this condition and its treatment. Two illustrative cases are presented.
Adolescent↗
Knee implant standardization: an implant selection and cost reduction program.
The largest single unit cost in the hospital cost for total knee arthroplasty (TKA) is the cost of knee implants. We developed a knee implant standardization program to provide guidelines for knee implant selection and to reduce the cost of knee implants for hospitals. Patients are assigned to demand categories based on five criteria: age, weight, expected activity, general health, and bone stock. Implants are assigned to demand categories based on an implant's projected capacity to handle the patient's projected demand. The program was applied retrospectively to 127 knee replacement operations performed on 93 patients during 1992. If this program had been in place, 8.4% of what was actually spent on knee implants for these 127 patients would have been saved. If the most expensive implants allowed in each demand category had actually been used, the program would have saved our hospital 12.8% of the cost of knee implants for these patients. Potential savings were noted in higher demand categories I and II by reducing the use of expensive cementless, porous-coated implants. The greatest potential savings were noted in lower demand categories III and IV: 11% savings could have been realized in demand category III, and 27% savings could have been achieved in the cost of knee implants in demand category IV. Potential savings would have been realized in these lower demand categories because of the recommended use of an all-polyethylene tibial component in 38 of 92 patients. This knee implant standardization program has the potential to assist surgeons in selecting knee implants and reduce the cost of knee implants without compromising outcome following TKA.
Aged↗
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.
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Clinical evolution in an outpatient series with indeterminate colitis.
PURPOSE: Diagnosis of indeterminate colitis, which is mainly based on histologic criteria, could represent either an interlocutory or a definite classification within inflammatory bowel diseases. A later evaluation could allow elimination of cases with transient attacks of colitis and the eventual change of diagnosis to that of ulcerative colitis (UC) or Crohn's disease of the colon in some other patients. METHODS: A clinical follow-up study for a mean of 64 months was performed in 37 patients with inflammatory bowel disease with an initial diagnosis of indeterminate colitis. RESULTS: At the end of the follow-up period, 21 patients complained of persistent symptoms, and in 13 of these patients, endoscopic and histologic evolution of colitis was controlled. In four patients with initially a normal endoscopy, the pattern of normality was confirmed also on a histologic basis at the end of the follow-up. In seven of the remaining nine patients with an initial UC-like endoscopic picture, the UC diagnosis was made eventually also on a histologic basis. CONCLUSIONS: A closer monitoring, as with UC patients, could be recommended only in moderate patients with indeterminate colitis, with an initial UC-like endoscopic picture.
Adolescent↗
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↗
Defective interleukin-2 production in children with chronic hepatitis B: role of adherent cells.
BACKGROUND: Chronic hepatitis B (CHB) virus infection is associated with functional abnormalities of cell-mediated immunity, defective interferons alpha and gamma synthesis, and interleukin-2 receptor expression. In this study, interleukin-2 (IL-2) production and the role of adherent cells was evaluated in 25 children chronically infected with hepatitis B virus. METHODS: IL-2 activity was measured by bioassay in supernatants of phytohemoagglutinin-stimulated peripheral blood mononuclear cells. In a few patients, IL-2 concentration was also immunochemically determined. Coculture experiments using a mixture of adherent cells and lymphocytes from healthy children and patients with CHB were also performed. RESULTS: Children with CHB showed lower IL-2 production than healthy controls. In patients, IL-2 activity was 34.7 +/- 22.5 U/ml as compared to 152.6 +/- 78.5 U/ml of controls. Immunochemical quantitation of IL-2 confirmed a lower IL-2 production in patients. No correlation was found between the functional T-cell defect and the severity of liver damage, degree of viral replication, and duration of the disease. In co-culture experiments, adherent cells from HBsAg-positive patients inhibited IL-2 production following mitogen stimulation of control non-adherent cells by 67%. The inhibitory effect, mediated by patients adherent cells, was abolished by blocking with indomethacin prostaglandins, that are potent local immunomodulators released by adherent cells. CONCLUSIONS: Our results further support the observation that in children with CHB virus infection adherent cells play an important role in the inappropriate regulation of immune response, an effect being likely mediated by prostaglandins.
Adolescent↗
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↗
[Hereditary non polyposis colon cancer (NHP CC) prevalence in South Italy (Campania). Report on a preliminary retrospective study].
The aim of this study was to determine a prevalence of Hereditary Non Polyposis Colo-rectal Cancer (HNPCC) in consecutive one hundred twenty-eight patients living in Campania district and affected by first diagnosed colorectal cancer. Data on 128 patients and their relatives was collected and available for analysys. Our preliminary results seem to demonstrate a low prevalence of HNPCC in Campania and will be verified with a prospective multicentric study in the same area.
English Abstract↗
Opportunities for control of hospital cost for total knee arthroplasty.
The hospital financial records of 120 consecutive patients who underwent unilateral knee replacement surgery at one hospital during 1995 were reviewed to determine opportunities for control of hospital cost for total knee arthroplasty. The average hospital length of stay for these patients was 4.27 days (range, 3-10 days). The average hospital cost was $10,231. All 120 patients were classified under Diagnosis Related Group 209, principle procedure 81.54 primary total knee arthroplasty. Medicare paid for 70% of the patients. All payers were profitable except Medicaid and one managed care organization. When hospital cost for total knee arthroplasty was allocated to hospital service centers, 78% of the cost was attributed to the operating room, nursing units, recovery room, and pharmacy. When hospital cost for total knee arthroplasty was allocated to hospital days, 80% of the hospital cost occurred during the first 48 hours of hospitalization. Hospital reimbursement for total knee arthroplasty is primarily a prospective case price payment system. After initial cost containment efforts reduce the hospital length of stay for total knee arthroplasty to 4 to 6 days, additional control of hospital cost should focus on these areas of opportunity.
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Lymphoblastoid interferon alfa with or without steroid pretreatment in children with chronic hepatitis B: a multicenter controlled trial.
The comparative efficacy of prednisolone followed by interferon alfa (IFN-alpha) versus IFN-alpha alone in enhancing the rate of antibody to hepatitis B e antigen (anti-HBe) seroconversion has not been evaluated in a large cohort of white children. To determine this, a multicenter-controlled trial was conducted in 95 hepatitis B virus (HBV)-DNA/hepatitis B e antigen (HBeAg)-positive children (median age, 9 years [range, 2-16 years]; 56 boys; 84 [89 percent] white), all having inflammatory changes on liver biopsy. Patients were randomized to receive either prednisolone followed by IFN-alpha (n = 34); placebo followed by IFN-alpha (n = 30); or no treatment (n = 31). The prednisolone/placebo was given on a double-blind basis. Lymphoblastoid IFN-alpha was given at 5 MU/m(2) three times a week for 12 weeks. Baseline clinical, biochemical, and histological features were similar for the three groups. The majority (85 percent) had a baseline aspartate aminotransferase (AST) level < or = 100 IU/L. On follow-up between 12 and 18 months (median, 15 months) after treatment, the loss of HBeAg with anti-HBe seroconversion was more common in patients pretreated with steroids (12 of 34 [35 percent]) or placebo [12 of 30 (40 percent)] as against controls (4 of 31 [13 percent], P< .05). Factors predictive of anti-HBe seroconversion were baseline HBV-DNA concentration of < or = 1,000 pg/mL and a greater degree of portal tract inflammation on pretrial biopsy. Our results show that in white children treatment with IFN-alpha, at the dose and duration used in this study, improves the rate of anti-HBe seroconversion. Steroid priming does not potentiate the effect of IFN-alpha.
Adolescent↗
Ursodeoxycholic acid for treatment of cholestasis in children on long-term total parenteral nutrition: a pilot study.
BACKGROUND & AIMS: Cholestatic liver disease (CLD) is a frequent and sometimes fatal complication of total parenteral nutrition (TPN) that may require withdrawal of TPN. The aim of this pilot study was to evaluate ursodeoxycholic acid (UDCA) as treatment of TPN-associated CLD. METHODS: Seven children (4 boys and 3 girls) undergoing long-term TPN because of intractable diarrhea syndrome developed cholestasis and were treated with UDCA. Treatment efficacy was evaluated by monitoring clinical and biochemical markers of CLD, including gamma-glutamyl transpeptidase (GGT), alkaline phosphatase (ALP), conjugated bilirubin, and alanine aminotransferase (ALT). RESULTS: In all children, UDCA was associated with the disappearance of signs of CLD and with normalization of biochemical markers of cholestasis within 4-8 weeks. A rebound increase of GGT, ALP, and ALT serum levels was observed in 3 children in whom UDCA was temporarily discontinued while they were still undergoing TPN. However, after reinstitution of UDCA, markers of cholestasis normalized in all cases. UDCA was withdrawn on reinstitution of full oral feeding; there was no relapse of cholestasis. Six children fully recovered. One child died because of the lack of vascular access. CONCLUSIONS: UDCA appears to be an effective treatment for TPN-related cholestasis in children.
Alanine Transaminase↗
Lymphoblastoid interferon alfa treatment in chronic hepatitis C.
Interferon is becoming the standard treatment in adults for chronic hepatitis C. Twenty one children with histologically proved chronic hepatitis C (10 boys, range 2.5-13 years), who were otherwise healthy, were enrolled in a randomised controlled study to test their response to interferon alfa. Eleven children were treated with lymphoblastoid interferon alfa (3 million units/m2) for 12 months; 10 children received no treatment. All had raised transaminases and positive antihepatitis C virus (HCV) antibodies and HCV-RNA. Alanine aminotransferase (ALT) serum levels became normal in five (45%) treated patients after a mean of three weeks (range 1-6 weeks) and no relapse had occurred by the end of follow up (30th month). Only one (10%) untreated patient had normal ALT serum levels from the 11th until the 30th month. Disappearance of serum HCV-RNA, persisting throughout the follow up period, was observed in the six children (five treated) whose ALT became normal. Biopsy specimens in treated patients showed a significant improvement in Knodell's score (median (SD) basal 9.0 (2.2); final 2.0 (0.4)). Interferon treatment was well tolerated in all. This study confirms the efficacy of interferon in children with chronic hepatitis C, not only by restoring normal ALT serum levels, but also viral clearance and histological amelioration of liver inflammation. Contrary to reports in adults no biochemical and virological relapses occurred in responder children.
Adolescent↗
Is alpha-interferon treatment useful in children with non-B, non-C chronic hepatitis?
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Child↗
Implant standardization for total hip arthroplasty. An implant selection and a cost reduction program.
Total hip arthroplasty (THA) has been targeted by the United States federal government for cost control because of its high cost and rising incidence in the aging population. The hospital cost for THA during the 1980s was controlled by utilization review and a reduction in the volume of services delivered for each THA. The single largest increase in the cost of THA during the 1980s was the cost of hip implants. The Lahey Clinic Hip Implant Standardization Program was developed to provide objective guidelines for hip implant selection. These guidelines are based on the demands a patients is expected to place on his or her hip prosthesis. Because not every patient requires an expensive high-demand hip prosthesis, the standardization program also has the potential to reduce the hospital cost for hip implants without compromising patient care. Patients are assigned to four demand categories based on five objective criteria: age, weight, expected activity, general health, and bone stock. Selection of the prosthesis in each of the four demand categories is intended to match the implant's capacity with expected patient demand. The standardization program was retrospectively applied to 103 THAs performed during 1991. Analysis of variance demonstrated that patient variables and demand categories were statistically significant groupings. The cost of hip implants would have been reduced by 25.7% with the Lahey Clinic Hip Implant Standardization Program. A prospective outcome study is required to determine the long-term validity of this standardization program.
Activities of Daily Living↗