[Liver histologic changes in children with type 1 of Crigler-Najjar syndrome]. / Gistologicheskie izmeneniya pecheni u detei s sindromom Kriglera-Nadzhara 1 tipa.

Background. Crigler-Najjar syndrome (CNS) is a rare genetic disorder found in less than 1 per 1.000.000 births. It happens as a result of an error in UGT1A1 enzyme which can cause high unconjugated bilirubin levels. OBJECTIVE: To describe liver histology changes in patients who have undergone liver transplantation. METHODS: This retrospective cross-sectional study was performed to evaluate the liver pathologies of patients with type 1 of Crigler-Najjar syndrome (CNS1). We analyzed medical records and liver histologic specimens of 53 children who were transplanted in Namazi Hospital Organ Transplant Center affiliated with Shiraz University of Medical Sciences between 2009 and 2019. We studied the tissue of the explanted liver, which was replaced by transplants. Most of the patients were less than 2 years old, with an average age of 1.7 years. The collected data were analyzed using SPSS 22 software. RESULTS: The prevalent pathology found in the liver of these patients was periportal fibrosis (96.2%). Cholestasis was the second common finding (94.3%) followed by pericentral fibrosis (86.7%) and ductal reaction (22.6%). A significant correlation was only present between phototherapy time and ductal reaction grade. CONCLUSION: Our results indicated a high prevalence of fibrosis of different grades among CNS 1 patients which bolds the necessity of histologic examination before considering treatments such as gene therapy or hepatocyte transplantation.

Tacrolimus and Sirolimus Once Daily Monotherapy Regimen as a Safe and Effective Long-Term Maintenance Immunosuppressive Therapy in Pediatric Liver Transplantation.

BACKGROUND: Long-term efficiency of attenuated immunosuppressive therapies is not well characterized in pediatric liver transplantation (LT). OBJECTIVE: To assess the efficiency of tacrolimus once daily (TAC-OD) and sirolimus once daily (SLR-OD) immunosuppression in pediatric LT. METHODS: We retrospectively evaluated 59 children who underwent LT in our center during 2002 to 2016. Those including children who underwent planned decrease in immunosuppressant dose (stable clinical conditions after 2 years of LT), and those who underwent unplanned decrease in immunosuppressant dose (because of complications such as post-transplant lymphoproliferative disorder [PTLD] and renal failure). RESULTS: 25 of 59 children underwent planned decrease in immunosuppressant dosage (mean±SD duration of 4.5±1.8, range: 3-11 years); 34 had unplanned decrease (mean±SD of 1.3±0.6, range: 0.5-2.6 years). 19 of 25 children with planned conversion received TAC-OD; 6 received SLR-OD (22 with 1 mg/day dose, and 3 with 1 mg every two days). Of 34 children with unplanned conversion, 27 received TAC-OD, 7 SLR-OD (25 children with 1 mg/day, 7 with 1 mg every two days, 1 with 0.5 mg/day TAC, and 1 with 0.5 mg TAC every two days). We found no adverse events including acute or chronic graft rejection, renal insufficiency, infections, PTLDs, or cardiovascular thrombotic events after initiation of the modified immunosuppression in none of the groups. CONCLUSION: TAC-OD or SLR-OD monotherapies are safe and effective for long-term management of LT children with either stable clinical conditions or those with LT complications.

Diagnostic Value of a Modified Version of Wilson's Diagnostic Score in Pediatrics.

Background: Wilson's disease (WD) is an autosomal-recessive hereditary liver disease affecting copper metabolism. OBJECTIVE: To test the diagnostic value of a questionnaire for the diagnosis of WD in pediatrics age group. METHODS: 70 children with biopsy-proven diagnosis of WD and 70 without WD were included in the study. A modified questionnaire with 4 items was used for the diagnosis of WD. The results were then compared to the definite diagnosis made by pathology (the gold standard test). RESULTS: The median (IQR) modified score in those with WD was 4 (4-5), significantly (p<0.001) higher than that calculated for the comparison group, which was 0 (0-1). The most appropriate cut-off value for the score was 2.5, corresponding to a sensitivity and specificity of 100%, and 98.6%, respectively. Using this cut-off value to classify 20 children with and without WD who underwent liver transplantation resulted in an accuracy of 100%. CONCLUSION: The modified scoring system is a sensitive and specific diagnostic tool for the diagnosis of WD in children. This is especially important in regions with limited access to specific laboratory tests for the diagnosis of WD.

Acute Hepatic Allograft Rejection in Pediatric Recipients: Effective Factors.

BACKGROUND: Acute cellular rejection (ACR), a reversible process, can affect the graft survival. OBJECTIVE: To evaluate the relation between ACR and clinical factors in recipients of allograft liver transplantation. METHODS: 47 recipients of liver were consecutively enrolled in a retrospective study. Their information were retrieved from their medical records and analyzed. RESULTS: Of the 47 recipients, 38 (81%) experienced acute rejection during 24 months of the transplantation. None of the studied factors for occurring transplant rejection, i.e., blood groups, sex, age, familial history of disease, receiving drugs and blood products, type of donor, Child score, and Child class, was not found to be significant. CONCLUSION: During a limited follow-up period, we did not find any association between ACR and suspected risk factors.

Acute Hepatic Allograft Rejection in Pediatric Recipients: Independent Factors.

BACKGROUND: Acute cellular rejection (ACR) has a reversible effect on graft and its survival. OBJECTIVE: To evaluate the relation between ACR and clinical factors in recipients of liver transplant allografts. METHODS: 47 consecutive liver recipients were retrospectively studied. Their data were extracted from records and analyzed. RESULTS: 38 (81%) of the 47 recipients experienced ACR during a 24-month follow-up. The rate of rejection was associated with none of the studied factors-recipient's blood group, sex, age, familial history of disease, drugs and blood products received, type of donor, and Child score and class. CONCLUSION: During a limited follow-up period, we did not find any association between ACR and suspected risk factors.

In vitro activity of five antifungal agents against Candida albicans isolates, Sari, Iran.

BACKGROUND AND PURPOSE: Candidaalbicans is the most common causative agent of candidiasis. Candidiasis management is dependent on the immune status of the host, severity of disease, and the choice of antifungal drug. Antifungals, specifically triazoles, are widely administered for the treatment of invasive fungal infections. Herein, we aimed to evaluate the invitro susceptibility of C.albicans isolates to fluconazole (FLZ), itraconazole (ITZ), voriconazole (VRZ), amphotericin B (AMB), and Caspofungin (CAS). MATERIALS AND METHODS: A total of 44 clinical strains of C.albicans were collected from 36 patients admitted to four hospitals in Mazandaran Province, Iran. The invitro antifungal susceptibility testing was performed based on the Clinical and Laboratory Standards Institute methods. RESULTS: Generally, 34 isolates were susceptible to all the five antifungal drugs, while four isolates were susceptible or susceptible dose-dependent (SDD) and six isolates were SDD or resistant to these antifungals. The lowest minimum inhibitory concentration (MIC; 0.016 µg/ml) belonged to AMB and the highest MIC was for FLZ )16 µg/ml). The lowest MIC (50 0.063 µg/ml) was related to ITZ and the lowest MIC (90 0.25 µg/ml) pertained to CAS, in addition , the highest MIC (50 1 µg/ml) and MIC (90 4 µg/ml) were for FLZ. Four of the isolates showed resistance to both FLZ and VRZ, separately, and five isolates were resistant to ITZ. Caspofungin showed potent activity against more than %95 of the C.albicans isolates. CONCLUSION: Overall, we reported %9.1 resistance to FLZ and VRZ ,%11.3 resistance to ITZ and AMB, and %4.6 resistance to caspofungin .Our finding is in agreement with previous observations proposing that C.albicans isolates develop resistance to some antifungal drugs such as FLZ since they are widely used as prophylaxis.

Breast cancer and associated factors: a review.

This article investigated different dimensions of breast cancer and its associated factors. It revealed that breast cancer was and continues to be among the most prevalent and growing malignant diseases among Iranian women in the past four decades. In this article, required information was collected through literature review and keyword (cancer, breast cancer, cell, gene, life quality, women, prevalence, productivity, age, obesity, alcohol, cigarette, menopause, genetic, Cytokine, and mortality) query in credible scientific websites such as SID, Google Scholar, and comprehensive portal of human sciences. This disease affects all physical, mental, and social aspects of women life. On the other hand, such factors as social and family supports during the illness can reduce its damages. Although, the [exact] etiology of breast cancer is unknown, its associated risk factors were identified. Such factors as aging, history of breast cancer in the family, specific changes in breast(s), gene changes, history of productivity and menopause, lack of physical activity, alcohol consumption, obesity, nutrition, race, and radiation therapy to chest are risk factors of breast cancer.

Is Helicobacter pylori infection a risk factor for childhood periodic syndromes?

BACKGROUND AND OBJECTIVES: Helicobacter pylori (H. pylori) infection has been assumed to have roles in various extra-digestive diseases. The current study was designed to evaluate the incidence of H. pylori infection in patients with cyclic vomiting syndrome and its possible role in the etiology of this disease. DESIGN AND SETTING: In this case-control study, 120 cases with diagnoses of cyclic vomiting or abdominal migraine who were registered at the Gastroenterology Clinic at Shiraz University of Medical Sciences from 2010 to 2013 were enrolled. MATERIALS AND METHODS: Primarily information regarding the patients' diseases were collected with a data gathering sheet, and fresh morning stool samples were collected from the patients and examined for H. pylori stool antigen with the H. pylori Ag EIA test kit. The results were compared with those of healthy children from the control group. RESULTS: A total of 120 patients with cyclic vomiting (47.5%) and abdominal migraine (52.5%) with a mean age of 7.1 ± 3.4 (range 2-16 years) and a male-to-female ratio of 1.6 were included. The HPs Ag tests were positive in only 7 (5.8%) patients in our case group, and the HPs Ag tests were positive in 13 (13%) of the children in the control group; this difference was statistically insignificant. CONCLUSION: Our study did not support H. pylori infection as an etiological factor in CV or AM.