Sclerosing angiomatoid nodular transformation of the spleen in a child with anemia: a case report and review of the literature.

BACKGROUND: Sclerosing angiomatoid nodular transformation of the spleen is a relatively rare benign vascular lesion in both adult and pediatric age groups with unclear etiopathogenesis and variable clinical presentations. Many benign and also malignant splenic masses could mimic sclerosing angiomatoid nodular transformation, both clinically and radiologically. Herein, we report our experience with a case of sclerosing angiomatoid nodular transformation in a 3-year-old girl. CASE REPORT: A 3-year-old Iranian girl presented with abdominal pain, back pain, and constipation for 2 weeks. She was being followed up by a pediatrician due to her short stature and persistent anemia. Physical examination showed stable vital signs, short stature, pallor, and a puffy face. Laboratory evaluation showed normochromic normocytic anemia with a normal reticulocyte count, ferritin, and hemoglobin electrophoresis. Radiologic assessments revealed a hypoechoic lesion in the spleen with high vascularity, clinically suspected to be lymphoma. She was operated on, and after partial splenectomy, pathologic evaluation of the spleen showed a solitary, well-demarcated, and unencapsulated dark mass. Microscopic examination revealed micronodular appearance composed of irregular-shaped vascular spaces lined by plump endothelial cells and surrounded by concentric collagen fibers, features in keeping with sclerosing angiomatoid nodular transformation. The patient's anemia was resolved after surgery, and no clinical or radiologic deficits were noted during the 10-month follow-up visits. CONCLUSION: Although sclerosing angiomatoid nodular transformation is exceedingly rare in children, it should be considered a differential diagnosis in pediatric splenic neoplasms with concurrent hematologic manifestations, such as anemia.

Splenic Lymphangioma Mimicking Lymphomatous Involvement: A Case Report with Review of the Literature.

Lymphangioma is a benign malformation of lymphatic vessels usually found in the head and neck areas or axilla. They may involve visceral organs with a lower percentage. Splenic lymphangioma is a rare tumor. This disease is often seen in children but may be diagnosed incidentally in adults. Most patients are asymptomatic, but in large and multifocal lesions, the patient may have some nonspecific symptoms such as abdominal pain, abdominal distention, nausea, vomiting, and loss of appetite. Physical examination may show no specific findings or detect palpable masses. The preoperative diagnosis of splenic lymphangioma is challenging. Histopathological evaluation and sometimes immunohistochemistry tests can result in a definitive diagnosis. In this study, we present an 18-year-old man, with Burkitt's lymphoma who underwent laparotomy and total splenectomy as a result of cystic lesions discovered accidentally during imaging with the final diagnosis of splenic lymphangioma after histopathological evaluation.

Prenatal diagnosis of diphallia in association with bladder exstrophy: a case report.

BACKGROUND: Penile duplication or diphallia is a rare congenital anomaly with unclear pathophysiological cause. Most cases of diphallia are reported postnatally; however, today with the use of a high-resolution ultrasound device, in-uterine diagnosis of many congenital anomalies is possible. CASE PRESENTATION: Herein we report a multiparous mother at 25 weeks of gestation who referred due to an abnormal cystic structure protruding from a large abdominal wall defect located below the umbilicus that was noted during a routine exam. Target scan revealed two penile-like protrusions with an empty scrotal sac and double bladder in an otherwise normal fetus, which was confirmed postnatally. Neonatal microarray study and karyotype were normal. CONCLUSION: Diphallia could be detected prenatally as an isolated anomaly, associated with caudal duplication syndrome, or as an exstrophy-epispadias complex. As this is a rare congenital anomaly, all sonographers should be familiar with prenatal ultrasound features and associated anomalies, an important issue in prenatal counseling with parents, delivery planning, psychological support of the family, and postnatal management.

Liver transplantation in glycogen storage disease: a single-center experience.

BACKGROUND: Glycogen storage diseases (GSDs) are inherited glycogen metabolic disorders which have various subtypes. GSDs of type I, III, IV, VI, and IX show liver involvement and are considered as hepatic types of GSDs. Thus, liver transplantation (LT) has been proposed as a final therapy for these types of GSD. LT corrects the primary hepatic enzyme defect; however, the long-term outcomes of LT in these patients have not been extensively evaluated so far. There are few reports in the English literature about the outcome of GSD patients after LT. There has been no report from Iran. The present retrospective study aimed to evaluate the long-term outcomes of eight patients with GSD types I, III, and IV who underwent LT in the affiliated hospitals of Shiraz University of Medical Sciences, from March 2013 to June 2021. During this period, there were no patients with GSD VI and IX identified in this center. RESULTS: The median time of diagnosis of the GSDs and at transplant was 1 year and 11 years, respectively. All eight transplanted patients were alive at the time of follow-up in this study. None of them required a re-transplant. All of the patients showed normalized liver enzymes after LT with no sign of hypoglycemia. CONCLUSIONS: LT is an achievable treatment for end-stage hepatic involvement of GSDs with a cure for metabolic deficiency. Our experience in these eight patients shows a favorable outcome with no mortality and no major complication.

A large undifferentiated sarcoma of the liver in a 13-year-old girl treated with anatomical resection: a case report and review of the literature.

BACKGROUND: Undifferentiated embryonal sarcoma of the liver (UESL) is a rare liver tumor accounting for 6-13% of primary liver tumors. Accurate preoperative diagnosis is difficult, with a rather high misdiagnosis rate. Herein, we reported a very large UESL treated with anatomical resection. Our case is amongst the largest pediatric UESLs in the literature. CASE PRESENTATION: Herein, we report a 13-year-old girl presenting with right upper quadrant abdominal pain, postprandial vomiting, and abdominal distention, in which radiographic imaging demonstrated a huge UESL (28 × 20 × 12 cm). The patient was treated with partial hepatectomy and the 5 kg tumor was removed. The patient was discharged in good condition, with no significant complaints in her follow-up. CONCLUSIONS: Although different treatment strategies have been reported for UESL cases, anatomical resection is still the main treatment approach, especially for large tumors.

Umbilical artery to common femoral artery (CFA) transposition, a novel technique for limb salvage in a newborn: a case report.

BACKGROUND: Cardiac catheterization is a course of action with a low rate of complication; however, the potential risk factors should be considered before the procedure. The risk of arterial complications increases in sick premature infants, especially in the first days of life. CASE: A four-day-old neonate with cyanotic heart disease (tricuspid atresia) was referred to our tertiary center for patent ductus arteriosus (PDA) stenting by cardiac catheterization. During catheterization, the stent escaped and was trapped in the left external iliac artery. Following the stent retrieval, the left external artery was disrupted entirely and caused pulse-less left lower extremity. The patient was immediately transferred to the operating room to repair the artery. Retracted ends of the artery had caused a 3-4 cm space between them. In this rare and emergency situation, the left umbilical artery was used to maintain the common femoral artery`s (CFA) blood supply. Therefore, an end to end anastomosis of the distal part of the left umbilical artery with the proximal part of the left CFA was done successfully and uneventfully. CONCLUSIONS: The complications of cardiac catheterization that lead to surgical repair are almost challenging, and adequate preoperative planning should be performed. In selective cases, the umbilical artery can be used to maintain the blood flow to the common femoral artery.

Clinicopathological study of hepatic mesenchymal hamartoma and undifferentiated embryonal sarcoma of the liver: a single center study from Iran.

BACKGROUND: Undifferentiated embryonal sarcoma of liver (UESL) and hepatic mesenchymal hamartoma (HMH) are two rare entities which mainly affect the pediatric population. The aim of this investigation was to provide a comprehensive overview of the clinicopathologic characteristics of the patients diagnosed with these two conditions in a tertiary referral center in Iran. METHODS: In this retrospective study patients diagnosed with UESL or HMH between 2012 and 2020 were studied. A comprehensive histopathologic evaluation of the cases along with immunohistochemistry evaluation using a panel of antibodies was conducted. Furthermore, clinical, paraclinical, and treatment data and follow up information was collected. RESULTS: A total of 16 patients (8 UESL, 8 HMH) were studied in this investigation. Patients with UESL had a significantly (p = 0.002) higher age at diagnosis compared with those with HMH. Histologically, UESL cases were characterized by anaplastic cells with eosinophilic cytoplasm and bizarre nuclei and frequent atypical mitosis and spindling in a myxoid stroma while disordered arrangement of hepatic parenchyma, bile ducts, and primitive mesenchyme was seen in HMH. Furthermore, small round cells and extramedullary hematopoiesis were seen in 2 UESL and 3 HMH cases, respectively. Concurrent HMH was also seen in two UESL cases. Immunohistochemistry panel showed positive staining for Vimentin, Glypican-3, Desmin, CD56, CD10, and BCL2 in UESL cases and immunoreactivity for Vimentin, HepPar 1, Glypican-3, SMA, CD56, BCL2, and CD34 in various components of HMH. CONCLUSIONS: In this study, the clinicopathologic features of UESL and HMH cases are presented. We also evaluated the utility of an immunohistochemistry panel in the diagnosis of these two rare entities and suggested novel markers. Our study corroborated the findings of previous investigations and expanded the clinicopathologic features of these two rare entities with diagnostic and potential therapeutic implications.

Pediatric liver and kidney transplantation in the era of COVID-19: a follow-up study from a tertiary referral center in Iran.

BACKGROUND: We aimed to evaluate the impact of COVID-19 pandemic on pediatric transplant outcomes and determine whether to continue pediatric transplant activity or not, and how policies intended our center has been effective in preventing COVID-19 among organ transplant recipients. METHODS: We conducted a single-center, retrospective, cohort study of hospitalized pediatrics after organ transplantation at Shiraz transplant center since March to August 2020. All liver and kidney transplanted children were included the study and their laboratory and clinical related COVID-19 characteristics were followed up till 3 months after transplantation during hospitalization period and then weekly by the transplant committee. RESULTS: Fifty-one patients underwent transplantation including 11 kidney and 40 liver recipients. The mean age of the pediatric cases was 6.72 ± 5.47 years. A total of 11 patients died due to post-transplant complications, while none of the patients presented any sign or symptoms in favor of COVID-19 in the hospital course after transplantation. Six transplants including 2 kidney and 4 liver were canceled when positive PCR tests were detected in their donors before the surgery. In the 3 months of follow up, two patients presented with symptoms including high grade fever, malaise, rhinorrhea, and GI symptoms. Both patients had two negative PCR, and no radiologic or laboratory results regarding COVID-19 were also detected. One had positive influenza PCR, while the second one had a positive serologic test for EBV; CT, computed tomography CONCLUSION: Transplant programs could continue their activities during the COVID-19 pandemic with specific case selection, accurate screening methods and following protective protocols.

Rapid Growth of Hydatid Cyst: A Pediatric Case Report.

Hydatid disease is a parasitic infection caused by Echinococcus granulosus with worldwide distribution. The most affected organs are liver and lungs, but it can be detected in any other organs as well. We reported a 5-yr-old boy from Shiraz, southern Iran in 2017 who presented with abdominal discomfort. Imaging revealed multiple liver hydatid cyst and a huge kidney hydatid cyst. This case showed the possible implication of rapid growth of multiple hydatid cyst as well as unusual organ presentation in the pediatric population.

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.

High accurate lightweight deep learning method for gesture recognition based on surface electromyography.

BACKGROUND AND OBJECTIVES: Surface Electromyography (sEMG) is used mostly for neuromuscular diagnosis, assistive technology, physical rehabilitation, and human-computer interactions. Achieving a precise and lightweight method along with low latency for gesture recognition is still a real-life challenge, especially for rehabilitation and assistive robots. This work aims to introduce a highly accurate and lightweight deep learning method for gesture recognition. METHODS: High-density sEMG, unlike sparse sEMG, does not require accurate electrode placement and provides more physiological information. Then we apply high-density sEMG, which, according to previous studies, leads to sEMG images. In this study, we introduce the Sensor-Wise method, which has a higher capability to extract features compared to the sEMG image method due to its high compatibility with the nature of sEMG signals and the structure of convolutional networks. RESULTS: The proposed method, because of its optimal structure with only two hidden layers and its high compatibility, has shown no sign of overfitting and was able to reach an accuracy of almost 100% (99.99%) when it was evaluated by CapgMyo DB-a database through 96 electrodes. Using this method, even with 16 electrodes, we were able to reach an accuracy of 99.8%, which was higher than the accuracies reported in the previous studies. Additionally, the method was evaluated by the CSL-HDEMG database, where the accuracy reached 99.55%. Previous studies either introduced expensive computational methods with overfitting or reported lower accuracies compared to this study. CONCLUSIONS: The Sensor- Wise method has high compatibility with the nature of sEMG signals and the structure of convolutional networks. The high accuracy and lightweight structure of this method with only two hidden layers make it a proper option for hardware implementation.

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.

Successful Surgical Separation of Thoraco-Omphalopagus Symmetrical Conjoined Twins in Iran: Two Case Reports.

Conjoined twins are derived from the division of a single fertilized ovum; a phenomenon accompanied with multiple congenital anomalies. Such twins are identical, of the same sex, and more likely to be female. Most twins die during the embryonic period, and only 18% survive longer than 24 hours. There are complex anomalies in thoraco-omphalopagus twins that makes them unlikely to live long enough to undergo separation. Treatment of this uncommon condition presents both surgical and anesthetic challenges. The management of rare anomalies is difficult even for skilled surgeons. Therefore, it is logical to use the knowledge gained from previous experiences. We herein present the first successful surgical separation of two pairs of thoraco-omphalopagus conjoined twins at the Pediatric Surgery Center of Namazi Hospital (Shiraz, Iran). In both cases, the neonates had separate hearts and common pericardium. Contrast-enhanced computed tomography of two sets of twins showed fusion of sternum, pericardium, diaphragm, and left lobe of liver. Critical steps of the surgical separation were identified and contingency plans were made for possible partial liver donation and cross-circulation between twins. The separation procedure and reconstruction of the abdominal wall were successfully performed. Both pairs of twins, now 6- and 8-year-old, are healthy and have normal growth and are under follow-up.

Single Center Long-Term Results of Pediatric Liver Transplantation.

OBJECTIVES: Liver replacement continues to be the only definitive mode of therapy for children with end-stage liver disease. However, it remains challenging because of the rare donor organs, complex surgical demands, and the necessity to prevent long-term complications. Our objectives were to analyze 16 years of experience in the Shiraz University Organ Transplant Center. MATERIALS AND METHODS: We retrospectively analyzed the records of 752 patients (< 18 years old) who underwent orthotopic liver transplant at our center over a 16-year period. Mean age was 90 months, and male-to-female ratio was 1.25. Of the 752 transplants, 354 were whole organs, 311 were from living related donors, and 87 were in situ split liver allografts. Patient and graft survival rates were determined at 1, 3, and 5 years, and results between groups were compared. RESULTS: Overall mortality was 31.8%. The 1-, 3-, and 5-year patient survival rates were 77%, 69%, and 66%, respectively, whereas the respective graft survival rates were 75%, 68%, and 65%. We observed significant differences in survival according to graft type (log-rank test, P < .001). We also observed significant differences in survival probabilities according to age (log-rank test, P < .001). Cox regression was used to simultaneously analyze effects of age and graft type on survival. Both graft type and age significantly affected survival (P < .001). The 1-, 3, and 5-year survival rates for patients having whole organ transplants were 88%, 81%, and 78%. Patients who received living donor grafts had respective survival rates of 66%, 60%, and 58%, with rates of 65%, 47%, and 47% for patients who received split grafts. CONCLUSIONS: Our results were similar to those observed in the literature in terms of indication for transplant and posttransplant survival.

Liver Transplantation Status in Iran: A Multi-center Report on the Main Transplant Indicators and Survival Rates.

BACKGROUND: Iran's experience with liver transplantation (LT) began more than two decades ago. The purpose of this article is to present the status of LT in Iran, review specific characteristics of the programs, their outcomes, and their growth to become one of the largest LT programs in the world. METHODS: A questionnaire, asking for data on the number of transplants performed and specifics of the recipients and type of donors with focus on indications and outcomes was sent to LT programs. RESULTS: During a period of 23 years, 4,485 LTs were performed at 6 centers in the country. Of these, 4106 were from deceased donors and 379 were from living donors. There were 3553 adults and 932 pediatric recipients. Hepatitis B and biliary atresia were the most common etiologies in adult and pediatric patients, respectively. Overall survival rates at 1, 5, and 10 years were 85%, 77%, and 71% for adults and 76%, 67% and 56% for pediatric patients, respectively. CONCLUSION: Approval of the brain death law in Iran and coordinated efforts by the transplant centers to build comprehensive LT programs has resulted in the ability to procure more than 700 deceased donors per year with acceptable long-term survival.

Perforated Meckel's Diverticulum in a 3-day-old Neonate; A Case Report.

Perforation of Meckel's diverticulum is a rare complication in neonatal period. A 3-dayold term male neonate was transferred to our emergency room due to bowel perforation. Surgical exploration was done and perforated Meckel's diverticulum was detected. Pathological report of the tissue showed inflamed diverticulum with heterotopic gastric mucosa. This is the first report of Meckel's diverticulum perforation in a neonate in our country.

Nutcrackerlike Phenomenon Is An Unusual Cause for Gross Hematuria After a Kidney Graft.

Nutcracker phenomenon is the condition that occurs most commonly at the morphologic type by compression of the left renal vein between the aorta and superior mesenteric artery. The diagnosis is often delayed because of the variability in manifestations and absence of consensus on diagnostic criteria. We report a 30-year-old woman who presented gross hematuria several days after a kidney transplant. Nutcracker syndrome was established intraoperatively during open surgical approach for bladder clot evacuation. Renal repositioning was done with relief in the degree of hematuria intraoperatively. No episode of gross hematuria was observed on follow-up after 8 months.

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.

Prevention of Posttransplant Lymphoproliferative Disorder in Pediatric Patients With a Liver Transplant.

OBJECTIVES: This study sought to evaluate in pediatric liver transplant recipients the effects of hybrid antiviral therapy on the rate of posttransplant lymphoproliferative disorder. MATERIALS AND METHODS: All pediatric patients (87 cases) who had undergone a liver transplant between April 2011 and March 2012 took part in the study and received hybrid antiviral treatment (case group). Epstein-Barr virus polymerase chain reaction was monitored intermittently. The results were compared to those of a historical control group including 117 pediatric patients who received a liver transplant between April 2009 and March 2011. Follow-up was 27 to 47 months in the control group and 12 to 26 months in the case group. RESULTS: Posttransplant lymphoproliferative disorder occurred in 12 patients in control group (10.2%) and 5 patients in case group (5.7%) (P = .249). Of 12 cases of posttransplant lymphoproliferative disorder, death occurred in 5 cases in the control group (41.7%), while no posttransplant lymphoproliferative disorder-associated death was seen in the case group (P = .086). CONCLUSIONS: Although hybrid antiviral treatment did not result in a statistically significant decrease in posttransplant lymphoproliferative disorder and posttransplant lymphoproliferative disorder-associated mortality rates, considering the limited number of posttransplant lymphoproliferative disorder cases in this study, this decrease may be interpreted as noticeable, and we advise using this strategy for pediatric patients undergoing a liver transplant.

Liver Transplant for Children With Hepatocellular Carcinoma and Hereditary Tyrosinemia Type 1.

OBJECTIVES: This study sought to determine the prevalence of hepatocellular carcinoma and other premalignant lesions in children with hereditary tyrosinemia type 1 who had undergone an orthotopic liver transplant at the Shiraz Transplant Center, in Shiraz, Iran. MATERIALS AND METHODS: Between September 2006, and June 2011, thirty-six patients with hereditary tyrosinemia type 1 received a liver transplant from a deceased (whole or split) or a living-related donor. Clinical records and pathologic specimens, before and after surgery, for each case were reviewed. In addition, ultrasound, abdominal computed tomographic imaging scan findings, and levels of alpha-fetoprotein were recorded. RESULTS: Twenty-two patients with hepatic nodules larger than 10 mm underwent a Tru-Cut needle biopsy before their liver transplant. In 2 patients, a diagnosis of hepatocellular carcinoma was made by pathologic examination; in the other 20, cirrhosis was confirmed with no evidence of malignancy. After pathologic examination of the explanted livers, the largest nodules in the 36 patients were 35 mm. Five cases had at least 1 nodule of hepatocellular carcinoma. Three of the other patients had small cell dysplasia in some of nodules. All 5 cases with hepatocellular carcinoma were patients older than 2 years of age (19 patients were older than 2 years of age). All patients with hepatocellular carcinoma received pretransplant nitisinone treatment. All patients with hepatocellular carcinoma after their liver transplant are alive at the time of this writing. CONCLUSIONS: The prevalence of cell dysplasia and hepatocellular carcinoma in children with hereditary tyrosinemia type 1 in our study is not as high as that reported previously, so it appears that patients older than 2 years of age require a liver transplant.