[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.

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.

The Correlation between Serum Level of Leptin and Troponin in Children with Major Beta-Thalassemia.

BACKGROUND: Polypeptide hormone Leptin suppresses inflammation in the heart muscle and protects heart from diseases. The purpose of this study is to evaluate the relationship between leptin and troponin serum levels with cardiac involvement in patients with major beta thalassemia. MATERIALS AND METHODS: In this cross-sectional study, 70 children with major thalassemia were selected. Two ml blood was taken as sample from all children and after separating serum; the samples were maintained in -20°C temperature. Then, regarding cold chain conditions, the sample were sent to the Biochemistry Lab. Afterwards, leptin and troponin serum levels with the relevant kits and BMI were measured in all children, and information about age and gender was recorded. Collected data were analyzed with SPSS. RESULTS: The mean of leptin in girls and boys were 2.47 ± 3.13 and 0.96 ± 1.08 respectively which showed a significant difference (t=2.74, p =0.009). A significant correlation was also observed between BMI and leptin (r = 0.374, P = 0.002). Another significant association was found between leptin and age (r = 0.248, P = 0.041). However, a significant inverse correlation between serum ferritin and age (r = - 0.607, P = 0.0001) was discovered. No correlation was found between leptin, troponin, and ferritin. CONCLUSION: Since Leptin increases with the advent of cardiac involvement and independent from troponin T, it can be a predictive marker of cardiac involvement in patients with major beta thalassemia.

Correlation between serum leptin level and thyroid hormones in children with major beta-thalassemia .

BACKGROUND: Beta-thalassemia is the most common hematology disease in human and leptin is one of the hormone that produce by adiposities cells. The purpose of this study was to investigate the relationship between serum leptin level and thyroid hormones in children with major beta-thalassemia. MATERIALS AND METHODS: This descriptive-cross sectional study was performed on 90 children aged 6-16 years old with beta-thalassemia. Body Mass Index (BMI ) were meuseurd in all patients and then, after collecting the samples, leptin and thyroid hormones levels of the serum were measured in the patients with thalassemia via ELISA method. Then, all data was analyzed by Pearson correlation test, and x2 statistical tests and P < 0.05 was considered as a significant difference. RESULTS: The mean of body mass index and serum leptin level in the patients group was 16.58±2.43 and 1.521 ±2. 49, respectively. The mean serum levels of thyroxin (T4), triiodothyronine (T3), and thyroid- stimulating hormone (TSH) in patient's groups were7.94 ±3.56, 1.28 ± 0.46, and 2.85 ±3. 44, respectively. There was significant correlation between serum leptin levels and T4 in patients with major thalassemia; also there was no significant correlation between serum leptin level and T3and TSH. There was a significant correlation was between the leptin serum level and BMI in patients (P value=0.008). CONCLUSION: The results of this study demonstrated that in patients with major thalassemia, there was significant correlation between serum leptin level and thyroxin hormone. Leptin level has more relationship with thyroxin than thyroid- stimulating hormone.

A study of leptin serum concentrations in patients with major Beta-thalassemia.

BACKGROUND: The aim of this study was to evaluate leptin serum levels in patients with major beta thalassemia which was also associated with their ferritin serum levels. MATERIALS AND METHODS: This case-control study was performed on 90 children -6 months to 16 years old, in Zabol, Amir- al- Momenin Hospital. Patients were divided in two groups and were matched in age and sex. All Children were examined and those eligible children who had not known heart disease, iron deficiency anemia, kidney disease, diabetes, fever and systemic diseases were enrolled after taking the informed consent of their parents. After collecting the samples, leptin and ferritin levels of the serum were measured in two groups by ELISA method. Then, the data was analyzed by the related statistical tests and SPSS 20 software. RESULTS: The mean of the serum levels of leptin and ferritin showed a significant difference in the case and control groups (P-value<0.05). An inverse statistical correlation was found for the serum levels of leptin and ferritin among the studied groups (P-value<0.05). Levels of leptin in the case group showed a significant gender difference (P-value<0.05), while based on BMI and age, no significant difference was observed for the serum levels of leptin in the case group. CONCLUSION: Based on the results of this study, major thalassemia reduces serum levels of leptin regardless of age and body mass. The study also found an inverse statistical correlation between serum levels of leptin and ferritin among the studied people.

Prevalence of HPV Infection and High Risk HPV Genotypes (16, 18), among Monogamous and Polygamous Women, In Zabol, Iran.

BACKGROUND: This study was performed to compare the prevalence of HPV infection and high risk HPV genotypes [16, 18] between monogamous and polygamous women, in Zabol, Iran. METHODS: This cross sectional study was conducted in Zabol in 2006 - 2007. Two hundred sixty five married women attending the Gynecology Clinic for Cervical Disease Screening entered to this study. One hundred sixty two cases had monogamous, and 103 had polygamous husbands. HPV PCR samples were obtained from scrape of papsmear specimens. The biotinylated primers MY09/MY11, GP5+/GP6+, were utilized to enable amplification and detection of positive PCR products. Confirmation of HPV-16 and -18 were done by type-specific PCR primers HPV-16/F, HPV-16/R and HPV-18/F, HPV-18/R. RESULTS: Prevalence of HPV infection in monogamous and polygamous groups was 29% and 37.9%, respectively. The most HPV infection was found in 15-25 years group. The most prevalence of infection in monogamous group was HPV-18 and HPV-non16, 18 in 15-25 years, and HPV-16 in 26-35 years group. In polygamous group the most prevalent type was HPV-16, 18 in 15-25 years group. The most prevalent HPV-16 was seen in sever inflammation and dysplasia cytology in both groups. CONCLUSION: Prevalence of HPV infection in Zabol is high, and in women with polygamous husbands group is slightly more than monogamous. Screening for this infection must be recommended in this region of Iran.