Expression of miR-let7b and miR-19b in progressive familial intrahepatic cholestasis (PFIC) children.

BACKGROUND: MicroRNAs (miRNAs) are a group of small non-coding RNAs that bind to the target mRNA and regulate gene expression. Recently circulating microRNAs were investigated as markers of diseases and therapeutic targets. Although various studies analyze the miRNA expression in liver disease, these studies on PFIC are few. Progressive familial intrahepatic cholestasis (PFIC) is a rare liver disease with autosomal recessive inheritance. Most children with PFIC progress to cirrhosis and liver failure and consequently need to have a liver transplant. The aim of this study is the investigation of the miR-19b and miR-let7b expression levels in Iranian PFIC children. METHODS: 25 PFIC patients, 25 healthy children and 25 Biliary Atresia patients were considered as case and two control groups respectively. Blood samples were obtained and Liver function tests (LFTs) were measured. After RNA extraction and cDNA synthesis, quantitative PCR was performed using specific primers for miR-19b and miR-let7b. The U6 gene is used as an internal control. RESULTS: qPCR on PFIC patients' samples demonstrated that the miR-19b and the miR-let7b expression were significantly decreased in patients compared to the control groups, with a p-value<0.0001 and p-value=0.0006 receptively. CONCLUSION: In conclusion, circulating micro-RNA like miR-19b and miR-let7b have a potential opportunity to be a non-invasive diagnostic marker or therapeutic target for PFIC in the future.

Extra-intestinal manifestations of Celiac disease in children: their prevalence and association with human leukocyte antigens and pathological and laboratory evaluations.

BACKGROUND: Celiac disease (CD) is an autoimmune disease caused by gluten intake. Traditionally CD was believed to be a disease of the gut, although a wide range of extra-intestinal manifestations (EIM) was recognized. The exact prevalence of EIM and the associated risk factors have not been well studied. AIM: We aimed to assess the prevalence of EIM in children with CD and their association with human leukocyte antigen (HLA) typing, and pathological and laboratory indices. METHOD: We conducted a cross-sectional study on children and adolescents with a definite diagnosis of CD. They were followed in the main Celiac Clinic of Southern Iran. RESULTS: We included 204 children who were visited between 2012 and 2017. Nearly 85% of them were positive for HLA-DQ2 and 40.6% for HLA-DQ8. The most prevalent intestinal complaints reported were abdominal pain (42.6%) and chronic constipation (19.1%). Failure-to-thrive (32.7%), iron deficiency anemia (25%), short stature (20.5%), and eczema (18.6%) were the most common EIMs. However, failure-to-thrive and short stature were presented at significantly younger ages, whereas those patients with concomitant type 1 diabetes mellitus (DM) were significantly older. We also found significant relationships between autoimmune thyroid disease and HLA-DQ5, and the presence of headaches with HLA-DQ7. The prevalence of HLA types of DQ2, DQ8, DQ6, and DQ7 significantly varied among different Marsh groups. Patients who were positive for HLA-DQ8, were significantly older, taller, and weightier. No significant association was found between HLA types and any of the gastrointestinal symptoms, anti-tTG and compliance to gluten free diet. Moreover, there were no statistically significant differences detected between the presence of each individual EIM, the level of IgA anti-tTG, sex, and Marsh typing. CONCLUSION: This study highlights the presence of EIM in CD and their associated factors. We show the potential role of HLA typing in some EIMs, which may shed light for future studies.

Evaluation of Outcomes and Complications of Large Volume Paracentesis without Albumin and Coagulopathy Therapy in Pediatrics with Severe Ascites.

Background: Large-volume paracentesis has become the first treatment choice for patients with severe and refractory ascites. The studies have reported several complications after therapeutic paracentesis. But there are few published data on the complications with or without Albumin therapy. We aimed to analyze the safety and complications of large-volume paracentesis in children with or without albumin therapy. Methods: This study was conducted on children with severe ascites with chronic liver disease who underwent large-volume paracentesis. They were divided into albumin-infused and albumin non-infused groups. In the case of coagulopathy, no adjustment was made. Albumin was not administered after the procedure. The outcomes were monitored to evaluate the complications. To compare two groups, a t-test was utilized, and the ANOVA test was used to compare several groups. If the requirements for using these tests were not met, Mann-Whitney and Kruskal-Wallis tests were applied. Results: Decreased heart rate was observed in all time intervals and was meaningful six days after paracentesis. MAP also decreased statistically at 48 hours and six days after the procedure (P < 0.05). Other variables did not show any meaningful change. Conclusion: Children having tense ascites with thrombocytopenia, prolonged PT, Child-Pugh class C, and encephalopathy can undergo large-volume paracentesis without any complication. Albumin administration before the procedure in patients with low levels of Albumin (<2.9) can effectively overcome the problems of tachycardia and increased mean arterial pressure. There will be no need for Albumin administration after paracentesis.

An evaluation of ascitic calprotectin for diagnosis of ascitic fluid infection in children with cirrhosis.

BACKGROUND: The most common infection in children with the hepatic disease with or without cirrhotic ascites is spontaneous bacterial peritonitis (SBP), which occurs in the absence of an evident intra-abdominal source of infection. The present study aims to assess the value of calprotectin in ascitic fluid in the diagnosis of ascitic fluid infection in children with liver cirrhosis. MATERIALS AND METHODS: In this cross-section study, 80 children with underlying liver disease who attended the Hepatology and Emergency Department in Shiraz University Hospitals were studied. All the patients were evaluated by a thorough history, clinical examination, laboratory investigations, diagnostic paracentesis with PMNLs count, and Calprotectin, which was measured in 1 mL ascitic fluid by ELISA. RESULTS: Thirty-five patients (43.75%) were diagnosed with ascitic fluid infection. Of these children 6 cases had positive ascitic fluid culture (SBP). Calprotectin was high in AFI patients with a statistically significant difference in AFI patients compared to non-AFI patients. The cut-off levels were 91.55 mg /L and the area under the curve was 0.971. So it can serve as a sensitive and specific diagnostic test for detection of AFI in children with underlying liver disease. CONCLUSION: Elevated ascitic calprotectin levels in cirrhotic patients are a diagnostic and reliable marker for the detection of AFI and are considered a surrogate marker for PMN.

The Largest Single Center Report on Pediatric Liver Transplantation: Experiences and Lessons Learned.

OBJECTIVE: We described our experiences on pediatric liver transplantation (LT) from the largest LT center in the world termed the Shiraz Transplant Center. BACKGROUND: After the first successful pediatric LT in 1967, pediatric LT has become the routine treatment for children with liver failure worldwide. METHODS: Data on a total of 1141 pediatric cases of LT were collected. Specifics on baseline and anthropometric characteristics, clinicopathology, prognosis of recipients of LT, and donor characteristics are reported. RESULTS: Mean age of patients was 7.83 ±â€Š5.55 years old. Most common etiologies for LT were biliary atresia (15.9%), progressive familial intrahepatic cholestasis (13.4%), and Wilson's disease (13.3%), respectively.Whole organs, living donor grafts, and split grafts were used in 47.9%, 41%, and 11.1% of patients, respectively. In-hospital complications were seen among 34.7% of patients and the most common complications were infections (26.8%), bleeding (23.4%), and vascular complications (18%).Median (interquartile range) model for end stage liver disease score was 20 (15, 25). Main causes of death among patients were sepsis (35.2%), followed by post-transplantation lymphoproliferative diseases (10.5%), and primary nonfunction of liver (9%).Patient survival showed improvement over the years (1-year survival of 73.1%, 83.4%, and 84.4%, 2-year survival of 65.2%, 77.1%, and 78.7%, 5-year survival of 58.2%, 72%, and 77.8% for 1997-2007, 2007-2013, and 2013-2019, respectively; P < 0.001). CONCLUSIONS: This is the largest single-center report on pediatric LT in literature which provides valuable experiences in pediatric LT.

Concomitant BK virus infection and visceral Leishmaniasis in a pediatric liver transplant recipient.

BACKGROUND: Solid organ transplant recipients are vulnerable to various unusual infections. Visceral Leishmaniasis (VL) is a protozoal opportunistic infection, which may affect the immune-suppressed hosts and solid organ transplant recipients. The BK virus infection is an evolving challenge in kidney transplant recipients. However, there are very few reports of BK virus (BKV) nephropathy involving the native kidney in liver transplant recipients. To the best of our knowledge, this is the first report of the simultaneous occurrence of these rare infections in a liver transplant recipient. CASE REPORT: The patient was a 9-year-old girl, a case of liver transplantation who presented with the incidental finding of proteinuria, azotemia, and cytopenia. Investigations revealed that she had concomitant BKV nephropathy and visceral leishmaniasis. Both infections were successfully treated. CONCLUSION: BK virus should be considered as a cause of nephropathy in liver transplant recipients. The presenting features of fever, cytopenia, and splenomegaly in a post-transplant patient should remind of unusual infections such as VL other than the common post-transplant conditions.

Effect of COVID-19 on Kawasaki Disease: Decrease Age of Onset and Increase Skin Manifestation.

BACKGROUND: Kawasaki Disease (KD) is the most common childhood vasculitis and cause of acquired heart disease for no apparent reason. There is some evidence indicating infectious agents as possible triggers for KD. During the COVID-19 pandemic, vasculitis has been a presentation of COVID-19 in children. We performed this study to assess the association between KD and COVID-19. We evaluated KD hospitalized children during February to September 2020 for COVID-19 (group one) and compared their demographic, clinical, laboratory, and echocardiographic findings with KD patients from the same period time in 2019 (group two). We also compared the same data in COVID-19 positive and COVID-19 negative KD patients in 2020 pandemic period in Shiraz Namazi referral hospital at southwest of Iran. RESULTS: Thirty-two patients in group one compared with 44 patients in group two. Sixty-eight percent of group one KD patients were positive for COVID-19 during the pandemic period. KD Age of onset in the group one was lower than group two (4.38 years VS 5.5 years, P-value = 0.044). There was no difference in the demographic, clinical, laboratory, and echocardiographic features of the patients during and before the COVID-19 pandemic (p-value > 0.05). Moreover, Comparing COVID-19 positive and negative the incidence of rash was higher within COVID-19 positive cases (p < 0.05), and coronary artery abnormalities were more prevalent in COVID-19 negative cases (p < 0.05). CONCLUSION: Admission rate of KD was almost similar during the COVID-19 pandemic but 68% of KD admitted patient were COVID-19 positive. Age of onset for KD during the COVID-19 pandemic was lower and skin manifestation was higher than the same period time in last year.

Effects of partial internal biliary diversion on long-term outcomes in patients with progressive familial intrahepatic cholestasis: experience in 44 patients.

PURPOSE: Progressive familial intrahepatic cholestasis (PFIC) is a hereditary disease characterized by cholestasis, which may cause jaundice, severe pruritus, and cirrhosis in the later stages. By the invention of biliary diversion methods, these patients were prevented from undergoing liver transplant. Using biliary diversion techniques, the entero-hepatic cycle was interrupted. This lowers the bile acid pool and resolves the pruritus. Herein, we report 44 cases of PFIC who underwent partial internal biliary diversion (PIBD) and long-term follow-up of these children. This comprises the largest case series of PIBD. METHODS: All patients were diagnosed by liver biopsy as PFIC before the operation. All underwent cholecysto colic bypass by jejunal interposition due to severe pruritus unresponsive to medication. Laboratory blood tests, sonography, and physical exam were done before and after the operation once every 3 months. Besides, a questionnaire was designed to ask the patients about the symptoms after the operation, and a pruritus score was measured using the 5D-itch scale. RESULTS: 44 children (25 boys, 19 girls), between 1.75 and 27.5 years (at the time of this study) were followed for a median period of 54 months. Age at operation ranged from 2 months to 18 years, with a median of 29 months. Of these children, 14 were lost to follow up. Results showed a significant decrease in pruritus and sleep disturbance after the surgery (p < 0.001). Also, jaundice decreased from 82.1 before to 7.1% following the surgery. 50% of the patients became medication-free at follow-up. CONCLUSION: PIBD is a safe procedure which helps non-cirrhotic children preserve their liver function. Therefore, PIBD prevents them from undergoing liver transplant. Effective results were achieved in terms of severe pruritus and jaundice, and children were able to regain their sleep patterns. It also avoided external stoma, which is more convenient from the patient's point of view.

Clinical and laboratory data in pediatric autoimmune hepatitis: study from referral center in Iran.

BACKGROUND: Autoimmune hepatitis (AIH) is an auto-inflammatory liver disease of children and adults, affecting patients of any age, sex, race or ethnicity, with more prevalence in females. OBJECTIVE: The aim of this study was to evaluate clinical manifestation, laboratory findings, and outcome of children with autoimmune hepatitis. MATERIALS AND METHODS: We evaluated 86 patients treated and followed with final diagnosis of AIH between years 2010 to 2018. Physical findings (including jaundice, hepatomegaly, splenomegaly and encephalopathy), liver enzymes, liver histology and autoantibodies (including ANA, Anti LKM-1 and ASMA) were extracted from medical files. Then the patients were followed for their final outcome (including response to medical treatment or successful treatment withdrawal, liver transplantation or death). RESULTS: Among 86 patients with AIH with mean age 9.10±4.36 years old, 66.27% were females. Jaundice (75.6%) and hepatomegaly (46.5%) were the most frequent physical findings, followed by splenomegaly (32.6%) and encephalopathy (17.4%). Aminotransferases including AST and ALT were elevated at least 3 times more than upper limit of normal in most of the patients (61.6% and 55.81%, respectively). Autoantibodies were available in 53 of 86 patients, 24.5% had AIH-1, 3.8% had AIH-II and 67.9% were seronegative. Medical treatment including prednisolone and azathioprine was started for patients, 53 of 86 cases (61.6%) had remission and 11 of 86 (13.7%) tolerated medication withdrawal successfully. Among all cases, 26 (30.2%) patients needed liver transplantation. Mortality rate was 9 among 86 cases (10.5%). CONCLUSION: Jaundice and hepatomegaly was the most frequent clinical findings. Mortality rate was 10.5.

Complications After endoscopic balloon dilatation of esophageal strictures in children. Experience from a tertiary center in Shiraz - Iran (Nemazee Teaching Hospital).

BACKGROUND: Esophageal stricture is one of the most important complication of the caustic ingestion. OBJECTIVE: The aim of this study was to evaluate complications of balloon dilatation among children with esophageal stenosis. MATERIAL AND METHODS: In this retrospective study 82 children were included. Children who underwent balloon dilatation for esophageal stenosis were included in our study. Duration of study was 14 year starting from 2001. Mean age of the cases was 3.95±0.4 year (Min: 15 days, Max: 14 year). Chart review and telephone calling were the methods of data collection. Data was analyzed using SPSS. RESULTS: In this study, 47% of the patients were male and 53% of the cases were female. Caustic ingestion (33.7%) was the most common etiology for the esophageal stricture. Vomiting (87.8%) was the most common presenting symptom. Among our cases, 76.8% had no compliant after esophageal dilatation. Chest pain was the most common compliant after esophageal dilatation. Response rate was similar among boys and girls. Toddler age had the best treatment response after esophageal dilatation. CONCLUSION: Among our cases, 76.8% had no post procedural compliant after esophageal dilatation. Esophageal perforation was seen in 4.9% of the cases. Chest pain was the most common post dilatation complication.

Evaluation of frequency of autoimmune hepatitis autoantibodies in children with type 1 diabetes.

BACKGROUND: Diabetes mellitus type 1 (T1DM) is one of the childhood diseases with growing prevalence. Various accompanying autoimmune diseases were seen with type 1 diabetes. The most common autoimmune diseases with T1DM are autoimmune thyroiditis and celiac disease. In some reports, autoimmune hepatitis has been reported in association with DM-1. OBJECTIVES: The aim of this study was to evaluate autoimmune hepatitis autoantibodies in children with T1DM. MATERIALS AND METHODS: In this crosssectional study, 202 children with T1DM were evaluated (47.5% were males and 52.5% were girls). Liver enzymes, autoimmune hepatitis related autoantibodies such as anti-nuclear antibodies (ANA), anti-smooth muscle (ASMA) and anti liver and kidney microsomal antibodies (LKM-1) were measured. Liver ultrasound was done for participants and biopsy of liver was taken for children with increased echogenicity of the liver, hepatomegaly or elevated liver enzymes. Results analyzed by statistical software spss-16, Descriptive statistics and chi-square test, paired T-TEST. Level of less than 5% was considered statistically significant. RESULTS: In 6 patients ANA and in 4 patients (2%) ASMA was positive,1 patient was ASMA positive but ANA negative. None of the patients were Anti LKM-1 positive. 3 patients had positive ANA and ASMA, and increased liver echogenicity on ultrasound simultaneously. Histological evaluation was showed that 2 patients had findings in favor of autoimmune hepatitis. CONCLUSION: Auto antibodies were positive in 10 cases. ANA was positive in 6 (2.97%) of all cases. ASMA was positive in 4 (1.98%) cases. Increased echogenicity was found in 3 cases. Histological evaluation showed 2 patients had biopsy confirmed autoimmune hepatitis. AIH-2 was not seen among our cases.

Exercise Performance in Pediatric Liver Transplant Recipients and Its Related Cardiac Function.

The aim of this study was to evaluate an exercise test in pediatric liver transplant recipients and its relation to their cardiac function. This cross-sectional study was conducted on 58 children who had successfully undergone orthotopic liver transplantation at least 6 months prior to the study, with the same age and gender-matched control group. M-mode, Doppler, tissue Doppler echocardiography and an exercise test were performed for all the participants. The VO2 values and METS in patients were less than the control (P = 0.001). Left ventricular posterior wall thickness in systole, left ventricular posterior wall thickness in diastole, interventricular septum diameter in diastole, AT, pulmonary acceleration time, ST and EaT, AaM, and SS had a significant difference between patients and the control group (P value < 0.05). Maximal oxygen consumption (Max VO2) and metabolic equivalent task (METs) values had a significant correlation with tricuspid valve S parameter (P = 0.018, r = 0.310). Max VO2 and METs values did not have a significant correlation with the diastolic dysfunction index, such as E/A and E/Ea. In this study, the exercise test showed decreased functional capacity in liver-transplanted children; however, the echocardiographic evaluation did not reveal any definite correlation with systolic or diastolic dysfunction.

Use of complementary and alternative medicine among paediatric patients with hepatogastrointestinal diseases.

BACKGROUND: The use of complementary and alternative medicine (CAM) is becoming increasingly popular in Middle Eastern countries; it is often used for paediatric medical conditions such as chronic gastrointestinal complaints and failure to thrive. It is also commonly used in patients with chronic hepatic conditions such as cirrhosis and in hepatic transplant patients. AIMS: This study aimed to evaluate the prevalence, types and associated factors of CAM use in children with hepatogastrointestinal disease in Shiraz, Islamic Republic of Iran. METHODS: In a cross-sectional study carried out during September 2013-January 2014, 238 parents of children attending a paediatric gastroenterology and hepatology clinic at Shiraz University of Medical Sciences received a self-administered questionnaire covering associated factors of complementary and alternative medicine use; 210 questionnaires were completed and returned. RESULTS: Complementary and alternative medicine was used by 103 patients (49%); 74 (71.8%) of these used herbal remedies. Older age of the child significantly increased CAM use. Those with previous positive experience of CAM (62.1% of users, 40.2% of non-users) and those who had received recommendation for CAM by health care providers (24.3% of users, 6.6% of non-users) were significantly more likely to use CAM. CONCLUSIONS: This survey demonstrated the high prevalence of CAM use (especially herbal remedies) in paediatric patients with hepatogastrointestinal diseases.

Effect of sequential therapy on treatment of Helicobacter pylori infection in children.

BACKGROUND: Helicobacter pylori infection which plays a major role in the etiology of chronic gastritis and duodenal ulcers in children and adults is one of the commonest chronic infection worldwide. Cure of the infection leads to healing of gastric inflammation and prevention of peptic ulcer. OBJECTIVE: The aim of this study was to evaluate the efficacy of the sequential therapy for treatment of Helicobacter pylori infection. MATERIALS AND METHODS: In this study, 40 children with symptoms of H. Pylori that the infection was proved by endoscopy and biopsy and rapid urease test (UBT) were enrolled, and received sequential therapy (Lansoprazol, Amoxicillin) for 5 days and (Lansoprazol, Metronidazole and Clarithromycin) for next 5 days. The eradication rate of therapy was evaluated by stool antigen test 6 weeks after completion of therapy. This study was carried out in Pediatric Gastroenterology Clinic of Shiraz University of Medical Sciences, Shiraz, Iran. This study was approved by ethic committee of Shiraz University of Medical Sciences. RESULTS: Forty children with mean age of (10.8±4 years) were evaluated. The most common symptom on first admission was epigastric pain (82.5%), with mean duration of symptoms (16±14.5 month). The most common endoscopic findings was redness and erosion of the antrum (55%) and the most pathologic findings was chronic gastritis (77.5%). The most drug adverse effect was nausea (22.5%). The eradication rate of sequential therapy was 82.5%. CONCLUSION: Eradication rate of sequential therapy was 82.5% among our cases.

Percutaneous endoscopic gastrostomy in children: a single center study at Tertiary hospital Iran.

INTRODUCTION: The aim of this study was to evaluate complications after percutaneous endoscopic gastrostomy among children who underwent percutaneous endoscopic gastrostomy in Nemazee hospital. MATERIALS AND METHODS: All children who underwent percutaneous endoscopic gastrostomy were included in the current study. Place of the study was department of pediatric gastroenterology of Nemazee children hospital of Shiraz university of medical sciences. Duration of the study was 5 year starting from 2008. All drugs such as aspirin, NSAIDS, and heparin were discontinued 1-7 days before procedures. All patients were kept NPO 6-8 hours before procedure according to the age. Single dose antibiotic was prescribed for all cases before procedure. During procedure, all patients were sedated using propofol and or midazolam. Some patients required intubation. RESULTS: Of 39 cases who underwent PEG, 4 (10.2%) patients showed complication. The most common indication for PEG insertion were neurologic problem (84.6%) and metabolic disease (10.2%). Of our patients, 84.6% of the cases had the weight below third percentile. CONCLUSION: The most common indication for percutaneous endoscopic gastrostomy was cerebral palsy. The complication rate in our study was 10.2%. Celulitis was the most common complication.

The importance of TH22 and TC22 cells in the pathogenesis of Helicobacter pylori-associated gastric diseases.

BACKGROUND: An association exists between Helicobacter pylori (H. pylori), peptic ulcers, gastritis, and sometimes gastric carcinomas. Th22 cells have protective and inflammatory roles in defense against microbes. AIM: We investigated the frequencies of Th22, Tc22, Th22/17, and Tc22/17 cells in addition to the changes in levels of cytokines IL-22, IL-6, IL-23, TNF-α, IL-1ß, and TGF-ß in sera from patients with H. pylori-associated gastritis, and peptic ulcer, and in uninfected patients. METHODS: A total of 76 patients with H. pylori-associated disorders formed the studied group. Frequencies of T-cell subsets were determined by flow cytometry. Levels of cytokines IL-22, IL-6, IL-23, TNF-α, IL-1ß, and TGF-ß in the sera and supernatants of patients were measured by ELISA and flow cytometry. RESULTS: The study participants included 32 males and 44 females with a mean age of 38.5±15.3 years. We divided the infected group into peptic ulcer and gastritis (mild, moderate, active chronic, and chronic). The frequencies of Th22, Tc22, and Tc22/17 increased significantly in the peptic ulcer, moderate, active chronic, and chronic gastritis groups compared to the uninfected group. Th22/17 only increased significantly in the chronic gastritis group. We observed significant increases in IL-22 in the moderate and active chronic gastritis, IL-23 in the active chronic and chronic gastritis, and TNF-α in the peptic ulcer and moderate gastritis groups. Following in vitro antigenic stimulation, we observed significantly higher levels of IL-1ß, IL-23, and IL-6 in the active chronic gastritis group, as well as IL-6 and IL-1ß in the chronic gastritis group compared to the uninfected group. CONCLUSION: Increased Th22, Tc22, and Tc22/17 cells and IL-22 levels appear to be influential in progression and severity of H. pylori infection. Th22/17 can be an interesting therapeutic target for chronic H. pylori infections where eradication is more difficult.

Soft Palate Ulcer: An Unusual Presentation of a Posttransplant Lymphoproliferative Disorder.

Posttransplant lymphoproliferative disorder (PTLD) is a serious complication of solid organ transplantation. It has several clinical manifestations, including infectious mononucleosis-like syndrome, lymphadenopathy, and other organ involvement. Soft palate ulcer is a rare manifestation of PTLD. The aim of this study is to present a 17-year-old girl with liver transplantation due to metastatic liver disease. After transplantation, she presented with a painful whitish ulcerative lesion accompanied by central necrosis in the right soft palate area measuring about 3 cm×3.5 cm. Histopathologic evaluation of the lesion showed monomorphic B-cell-type PTLD. She received 6 courses of CHOP chemotherapy plus rituximab. In the follow-up, the lesion disappeared with scar formation. Therefore, PTLD has different clinical presentations and may involve any part of the body. Therefore, when an unusual oral lesion after solid organ transplantation is observed, PTLD should be considered in addition to other differential diagnoses.

Liver transplantation as a definitive treatment for familial hypercholesterolemia: A series of 36 cases.

FH is a genetic disorder characterized by an increase in serum LDL and total cholesterol values. The afflicted patients are at increased risk of premature atherosclerosis and myocardial infarction. Different treatment modalities are present, including pharmacological agents and surgical procedures. The most effective method of therapy in refractive cases is liver transplantation. Herein, we report our experience on 36 cases of patients with FH undergoing liver transplantation in our center, the main referral center of liver transplantation in Iran. The clinical findings, hospital courses, post-operative complications, and patient follow-up are also described.

A Comparison of the Effect of Kegel Exercises and Conventional Therapy versus Conventional Therapy Alone in the Treatment of Functional Constipation in Children: A Randomized Clinical Trial.

Background: There have been few studies on the effect of Kegel exercises on the treatment of functional constipation in children. Hence, the present study investigated the add-on role of Kegel exercises in children with functional constipation. Methods: This clinical trial was conducted on children with functional constipation, according to Rome IV, who were referred to the pediatric department of Imam Reza Clinic (Shiraz, Iran) in 2022. The sample consisted of 64 children who were randomly assigned to either the intervention or the control groups. In the control group, a pediatrician administered conventional therapy, including diet training, defecation training, and polyethylene glycol (PEG) syrup (0.7 g/Kg daily). In the treatment group, in addition to conventional therapy, a pediatrician taught Kegel exercises to the child both verbally and in writing in the presence of their parents. To investigate the effectiveness of the intervention, frequency of defecation, defecation time, assistance used for defecation, incomplete emptying, unsuccessful defecation, abdominal pain, and painful defecation were selected as the outcomes. Independent sample t test was used for continuous variables. Categorical variables were reported as frequency and percentages. To examine the difference in categorical outcome variables, Wilcoxon (pre and post), Chi square, and Fisher exact tests were used. Data were analyzed using SPSS software version 21. P<0.05 were considered statistically significant. Results: Twenty-seven (88.4%) patients in the Kegel exercise group reported a defecation time of less than 5 min, while only 12 (37.5%) patients in the control group reached this time, and this difference was statistically significant (P=0.001). Moreover, patients in the treatment group showed significant improvements in terms of incomplete emptying of stool, unsuccessful defecation, abdominal pain, and painless defecation (P=0.001, P=0.001, P=0.001, P=0.037, respectively). After intervention, the use of laxatives, digits, or enemas to assist defecation was not significantly different between the groups (P=0.659). Conclusion: Kegel exercise was an effective adjunctive treatment for pediatric functional constipation.Trial Registration Number: IRCT20230424057984N1.

Comparison of Mir122 expression in children with biliary atresia and healthy group.

Biliary atresia (BA) is the primary cause of neonatal jaundice with various pathological mechanisms. Many BA patients may experience progressive liver dysfunction and eventually need a liver transplant. Therefore, identifying potential non-invasive biomarkers for BA is crucial. miR-122, the most abundant microRNA in the liver, plays significant roles in different liver diseases. This study aimed to assess miR-122 levels in BA patients. Eighteen patients with biliary atresia were selected at random from the Shiraz Pediatric Liver Cirrhosis Cohort Study (SPLCCS), along with 18 healthy controls. Blood samples were collected, and biochemical parameters (such as liver function tests) were measured. Quantitative reverse-transcription PCR (RT-PCR) was conducted on serum samples from both the case and control groups to analyze miR-122 levels. The study results indicated that serum miR-122 expression in BA patients was elevated compared to the control group, although it did not reach statistical significance. Additionally, no correlation was found between miR-122 expression and serum levels of liver enzymes or other laboratory findings in BA cases. miR-122 could be a potential target for diagnosing BA; however, further research with a larger population is necessary to determine if miR-122 could serve as a useful biomarker for diagnosing BA.