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Background: Early diagnosis is mandatory for the medical care of children and adolescents with pediatric-onset inflammatory bowel disease (PIBD). International guidelines ('Porto criteria') of the European Society for Pediatric Gastroenterology, Hepatology and Nutrition recommend medical diagnostic procedures in PIBD. Since 2004, German and Austrian pediatric gastroenterologists document diagnostic and treatment data in the patient registry CEDATA-GPGE on a voluntary basis. The aim of this retrospective study was to analyze whether the registry CEDATA-GPGE reflects the Porto criteria and to what extent diagnostic measures of PIBD according to the Porto criteria are documented. Methods: Data of CEDATA-GPGE were analyzed for the period January 2014 to December 2018. Variables representing the Porto criteria for initial diagnostic were identified and categorized. The average of the number of measures documented in each category was calculated for the diagnoses CD, UC, and IBD-U. Differences between the diagnoses were tested by Chi-square test. Data on possible differences between data documented in the registry and diagnostic procedures that were actually performed were obtained via a sample survey. Results: There were 547 patients included in the analysis. The median age of patients with incident CD (n = 289) was 13.6 years (IQR: 11.2–15.2), of patients with UC (n = 212) 13.1 years (IQR: 10.4–14.8) and of patients with IBD-U (n = 46) 12.2 years (IQR: 8.6–14.7). The variables identified in the registry fully reflect the recommendations by the Porto criteria. Only the disease activity indices PUCAI and PCDAI were not directly provided by participants but calculated from obtained data. The category 'Case history' were documented for the largest part (78.0%), the category 'Imaging of the small bowel' were documented least frequently (39.1%). In patients with CD, the categories 'Imaging of the small bowel' (χ2 = 20.7, Cramer-V = 0.2, p < 0.001) and 'Puberty stage' (χ2 = 9.8, Cramer-V = 0.1, p < 0.05) were documented more often than in patients with UC and IBD-U. Conclusion: The registry fully reproduces the guideline's recommendations for the initial diagnosis of PIBD. The proportion of documented diagnostic examinations varied within the diagnostic categories and between the diagnoses. Despite technological innovations, time and personnel capacities at participating centers and study center are necessary to ensure reliable data entry and to enable researchers to derive important insights into guideline-based care. [ABSTRACT FROM AUTHOR]

Fifteen patients with significant coronary artery disease confirmed by cardiac catheterisation were studied before and after aortocoronary artery bypass graft surgery by rest and exercise 201Tl myocardial scintigraphy in an attempt to predict graft status non-invasively. Segmental myocardial perfusion comparisons in pre- and postoperative exercise images allowed correct predictions of high (greater than or equal to 67%) or low (less than or equal to 50%) graft patency rates in 10 of 15 patients, while similar comparisons in postoperative rest and exercise images yielded 13 of 15 correct predictions. Furthermore, 20 of 21 myocardial segments with increased postoperative perfusion when compared with corresponding segments in preoperative exercise studies were supplied by patent grafts. Regional graft occlusion, however, was difficult to predict. It was also difficult to predict graft status when myocardial segments were shown to have unchanged perfusion postoperatively as compared with the preoperative segmental assessment. Despite these limitations, the non-invasive technique of 201Tl imaging can provide useful information regarding coronary artery bypass graft patency. [ABSTRACT FROM PUBLISHER]

Aims: To study the effects of routine HLA screening and the policy of avoiding donor-dominant one-way HLA match to prevent graft-versus-host disease (GVHD) after living donor liver transplantation (LDLT)., Patients and Methods: The records of potential living liver donors and recipients who attended our center between 2007 and 2018 were reviewed retrospectively., Results: Of the 149 patients who underwent LDLT and survived longer than 3 months, two developed GVHD despite our strict policy. The first patient presented with grade II GVHD limited to the skin. She was treated successfully by briefly discontinuing immunosuppression and switching to everolimus. In the second case, the policy had been relaxed due to the availability of a single donor for ABO-incompatible transplantation without any intervention to decrease anti-A antibody levels (special case: A2 to O). Nevertheless, the patient presented with grade I GVHD limited to skin and was treated successfully by adding oral methylprednisolone to tacrolimus and mycophenolate mofetil. To the best of our information, this is the second reported case who recovered from GVHD after LDLT from a donor, homozygous at HLA A, B and DR and a recipient, heterozygous for all. Sixteen potential donors (1.2% of all candidates) of 14 recipients were disqualified solely on the basis of the HLA results; five of these patients died due to unavailability of another donor., Conclusion: The results support the policy of avoiding HLA combinations that preclude immune recognition of graft lymphocytes as foreign to decrease the risk of GVHD after LDLT., (© 2024 Wiley Periodicals LLC.)

Background: Exclusive enteral nutrition (EEN) is the first-line therapy for pediatric-onset Crohn's disease (CD) patients. CEDATA-GPGE® is the largest patient registry for children and adolescents with inflammatory bowel disease (IBD) in Europe, collecting data from over 5000 patients since 2004 in Germany and Austria. Since the application of EEN over 8 weeks is difficult and a high dropout rate is often described, the mode of application including a supporting structure is crucial for success. The aim of this study was to ascertain the variation in the application of EEN across the participating centers and to associate these with the outcome., Results: Thirty-one centers responded to the survey (81.6%). 88.5% of CD patients were recommended EEN for induction therapy, 71.8% actually started with EEN, and 22.1% terminated the EEN prematurely. The duration of EEN typically lasted 6 to 8 weeks, and the polymeric formula was mainly used. 80.6% of the clinics added flavorings to the formulas. After EEN, the most preferred diet for maintenance therapy was a healthy, well-balanced diet considering individual intolerances., Conclusions: EEN is widely recommended as an induction therapy by the German and Austrian pediatric gastroenterologists for children and adolescents with CD. However, this questionnaire-based study has shown a wide variation in EEN protocols used by the different pediatric clinics of CEDATA-GPGE®., (© 2022. The Author(s).)

Background: Living donor liver transplantation may complement cadaveric transplantation in acute liver failure (ALF) patients., Methods: Between 2008 and 2017, 89 patients were treated for ALF; 15 patients (17%) recovered with intensive care treatment; 31 (35%) died without transplant. The records of the remaining 43 patients (median (range) age: 14 (1-62)) who underwent transplantation were evaluated., Results: The etiologic factors were toxic agents (10; mushrooms: 8; herbs: 2), hepatitis viruses (7; A: 1; B: 6), Wilson's disease (7), autoimmune hepatitis (4), and Budd-Chiari syndrome (2); 13 cases were idiopathic. Cadaveric organs (whole, split, reduced) were transplanted to 32 patients; 11 patients underwent living donor transplantation. One patient (2%) died of septic shock on the second postoperative day. Bacterial infection was the most common early (< 3 months) complication in the remaining patients (31/42; 74%), followed by delirium (5/42; 12%) and acute rejection requiring steroid pulse (5/42; 12%). Seven other patients died during median (range) follow-up of 94 (14-142) months: various infections (5), leukemia (1), and acute myocardial infarction (1). The 1-, 5-, and 10-year survival rates were 100%, 96%, and 92% in children and 94%, 82%, and 65% in adults respectively., Conclusions: Cadaveric organ sharing and transplantation from living donors when appropriate yield a high survival rate, despite high early morbidity, in ALF patients whose conditions deteriorate despite intensive care treatment. Efforts to eliminate preventable causes of acute liver failure will lead to more efficient use of health care resources., (© 2021. The Society for Surgery of the Alimentary Tract.)

The biological role of the lipopolysaccharide-responsive beige-like anchor (LRBA) protein associated with the immune system is not to date well known. However, it is thought to regulate the CTLA4 protein, an inhibitory immunoreceptor. Chronic diarrhea, autoimmune disorders, organomegaly, frequent recurrent infections, hypogammaglobulinemia, chronic lung manifestations, and growth retardation are some features of LRBA deficiency. This rare disease is observed as a result of homozygous mutations in the LRBA gene. An 11.3-year-old male patient presented because of short stature and high blood glucose level. He had a previous history of lymphoproliferative disease, chronic diarrhea, and recurrent infections. His parents were first-degree consanguineous relatives. A diagnosis of type 1 diabetes mellitus (T1DM) was added to the preexisting diagnoses of immunodeficiency, recurrent infection, enteropathy, chronic diarrhea, lymphadenopathy, hepatomegaly, and short stature. Genetic analysis revealed a homozygous mutation in the LRBA gene, c.5047C>T (p.R1683*) (p.Arg1683*). Abatacept treatment was started: the patient's hospital admission frequency decreased, and glucose regulation improved. At follow-up, growth hormone (GH) deficiency was diagnosed, although it was not treated because the underlying disease was not under control. Nevertheless, the patient's height improved with abatacept treatment. LRBA deficiency should be considered in the presence of consanguineous marriage, diabetes, immunodeficiency, and additional autoimmune symptoms. LRBA phenotypes are variable even when the same variants in the LRBA gene are present. Genetic diagnosis is important to determine optimal treatment options. In addition to chronic malnutrition and immunosuppressive therapy, GH deficiency may be one of the causes of short stature in these patients.

Background/aims: To investigate the safety and long-term results of endoscopic retrograde cholangiopancreatography (ERCP) in children with a literature review., Materials and Methods: All patients within the age range of 6-17 years who underwent ERCP between 1994 and 2014 at our institution were retrospectively evaluated., Result: Twenty-four patients with a median age of 15 years underwent ERCP. Cannulation of the papilla was achieved in all patients (100%) without the use of needle-knife papillotomy. Before 1999, ERCP was used as a diagnostic method only in 7 patients (29%). In 17 (71%) patients, the procedure was used for therapeutic purposes. The indications were choledocholithiasis (10 cases, 42%), postoperative complications (5 patients, 21%), and recurrent pancreatitis (2 cases, 8%). In 2 patients (8%), the therapeutic effect was not achieved, thus requiring subsequent operations. There were no major complications. Mild pancreatitis occurred in only 1 patient (4%). Long-term follow-up information was obtained in 16 (67%) patients (median, 18 years; range, 3.5-22.5 years), and no long-term complications were detected., Conclusion: Endoscopic retrograde cholangiopancreatography is a valuable tool in the diagnosis and treatment of pancreatobiliary disorders in the pediatric population. Large-scale studies are required to create evidence-based guidelines specific to children.

Background: Juvenile Dermatomyositis (JDM) is a pediatric vasculopathy characterized primarily by skin and muscle involvement. Cardiac findings have been reported in children with JDM but have rarely been investigated in detail., Methods: We aimed to describe the relevant clinical and laboratory cardiac findings of a cohort of patients with JDM, followed at one centre, at disease diagnosis., Results: We performed a retrospective review of 105 patients with JDM, followed from 1991 to 2007. Six of 70 patients (9%, 6% of the entire cohort) had abnormal electrocardiographic (ECG) findings, while 26 of 54 patients (48%, 25% of the entire cohort) had abnormal echocardiographic (echo) findings. Many of these findings were either mild or unlikely to be a result of JDM., Conclusions: Our findings suggest that cardiac abnormalities at JDM disease onset are frequently seen, but are rarely significant findings due to disease; however, JDM patients should be considered for screening for cardiac disease as late cardiac complications are well recognized.

In this study, we report our experiences on the role of transplantation in 2 patients with large liver tumors in the setting of Abernethy malformation. Patient 1 was a 17-year-old boy who was referred for hepatic masses and recurrent hepatic encephalopathy episodes. Computed tomography and magnetic resonance imaging showed 2 large tumors (4 and 8 cm) in the liver. The portal vein drained directly into the vena cava. Core biopsy of the larger mass revealed fibrosis and regenerative hyperplasia. There were hyperintense signals in the T1-weighted images in the globus pallidus. The Stanford-Binet intelligence scale showed moderate mental retardation (IQ 39); however, the patient showed good ability for caring for himself. His cognitive defect was ascribed partially to chronic encephalopathy. The patient received a right hepatic lobe from his older brother. The congenital portacaval shunt was disconnected to provide inflow to the graft. Pathologic examination of the explanted liver revealed no evidence of malignancy. His IQ improved to 75 at 29 months posttransplant. The hyperintensity of the globus pallidus on magnetic resonance imaging disappeared. The patient has maintained a normal life during 9 years of follow-up. Patient 2 was a 17-year-old girl who was referred for multiple hepatic masses; she had no symptoms at admission. Magnetic resonance imaging showed type 1 Abernethy malformation and multiple hepatic masses (largest was 10 cm), which appeared to be hyperplastic lesions. Because malignancy could not be definitely excluded, she received a right lobe without the middle hepatic vein from her uncle. Pathologic examination of the explanted liver showed localized nodular hyperplasia; there was no evidence of malignancy. She has maintained normal life activities during 3 years of follow-up. Liver transplant is a curative treatment option for patients with large liver tumors, replacing the hepatic parenchyma in the setting of Abernethy malformation.

Background: There are still ongoing controversies about several aspects of lymphatic mapping and sentinel lymph node biopsy for breast cancer, including injection site of radioisotope and blue dye. This study aims to evaluate the success rate of different radiocolloid injection techniques in the detection of sentinel lymph nodes (SLN) in early breast cancer., Study Design: One hundred ninety-two women with early breast cancer were included. For SLN mapping with lymphoscintigraphy (LSG), 5 different injections were used. Group A (36 patients) had 4 peritumoral (PT), group B (n = 36) had 1 subdermal (SD) injection of Tc-99m rhenium sulfide colloid over the tumor quadrant. Group C (59 patients) had 1 PT and 1 SD combined injections. In group D (56 patients), lymphatic mapping was performed with 2 intradermal periareolar (ID-PA) injections. In group E (n = 41), 2 ID-PA and 1 PT combined injections were performed. Early dynamic and delayed images were obtained. A surgical gamma probe was used to explore the SLNs. Surgical specimens were evaluated histopathologically. The SLN identification rate, false negative rate, and comparison of groups were evaluated by statistical methods., Results: The SLN identification rate by LSG in groups A, B, C, D, and E were 72%; 92%, 93.2%, 98%, and 95%, respectively. The highest detection rates for the axilla (98%) and mammary internal (MI) drainage (22%) were obtained with ID-PA injections and a peritumoral injection, respectively. Seventy of 192 patients (36.4%) had positive axillary lymph nodes. The only statistically significant difference was between the PT and SD injection groups in axillary SLN identification rate by LSG (P = 0.016)., Conclusion: The success rate was superior with intradermal periareolar injection compared with PT and SD injection to visualize the axillary SLN. However, PT deep injection combined with ID-PA injections may be more favorable to demonstrate the primary internal mammary (IM) lymphatic drainage.

Purpose: The aim of this study was to evaluate the success rate of combined peritumoral and subdermal injection techniques based on our previous experience on different injection techniques for lymphoscintigraphy., Patients and Methods: Fifty-nine women with early breast cancer (mean tumor size, 20.5 mm) were prospectively studied. On the morning of the operation, each patient had 2 injections, one peritumoral (PT) medial to the lesion and one subdermal (SD) into the skin over the tumor quadrant. Each injection consisted of 20 MBq (540 mCi) Tc-99m rhenium sulfide colloid. Early dynamic and delayed static images were obtained up to 4 hours after injections. An intraoperative gamma probe was used to explore the axillary sentinel lymph nodes (SLN). All surgical specimens were evaluated histopathologically., Results: Forty patients had breast-preserving surgery and 19 had modified radical mastectomy. Thirty-eight patients had axillary dissection. All but 4 patients showed axillary lymphatic drainage. Twelve of 59 patients (20%) showed extraaxillary drainage with lymphoscintigraphy. Combined injection technique yielded a 93.2% success rate in detecting axillary SLN. In 2 of 4 patients with no drainage on lymphoscintigraphy, intraoperative gamma probe revealed SLN during the surgery. Twenty patients (33%) had positive axillary lymph nodes. In 14 of them, the SLN was the only positive node. A false-negative rate was found 1.6% (one of 59 patients)., Conclusion: This results suggest that a combination of both PT and SD techniques increases the success rate of visualization SLN and enhances the visualization of extraaxillary nodes for further treatment planning.

Primary melanoma of the anal region is a rare pathological entity and its prognosis is generally poor. The aim of this report is to demonstrate the feasibility of the sentinel lymph node (SLN) procedure with combined technique in patients with anal melanoma. We report of two cases with anal melanoma that had wide local excision of the primary lesion and was referred for further evaluation. After diagnostic metastatic work-up, SLN procedure consisted of a combination of preoperative lymphoscintigraphy with technetium-99m nanocolloid injected around the tumor, and intraoperative detection of SLN with gamma probe (combined technique) was performed. In addition, patent blue dye was injected at the periphery of the tumor to facilitate direct identification of the blue-stained lymph node. In the first case, SLN identified both inguinal and iliac lymph node basins, both of which were histologically negative on both frozen and paraffin sections. In the other case, SLN removed from the inguinal lymph node basin showed micrometastasis by paraffin section. In both cases SLN procedure with combined technique was performed sufficiently without significant complications. Consequently, we suggest that SLN procedure with combined technique is also a useful technique in malignant melanomas similar to other anal canal cancers.

While continuous ambulatory peritoneal dialysis (CAPD) offers several advantages over hemodialysis in patients with end-stage renal disease, several complications have been recognized. The intraperitoneal instillation of dialysate increases intra-abdominal pressure and consequently predisposes the patient to leaks and herniations through defects in the abdominal wall. The use of an intraperitoneal radiolabeled colloid has been previously described to image entities such as hernias, patent processus vaginalis, abdominal wall, and diaphragmatic leakage. This study shows a simple, non-invasive method of determining the site of dialysate leak and its importance to assist further patient management. There has been a continuing increase in the number of end stage renal disease patients maintained on chronic peritoneal dialysis (CPD). Many patients choose CPD as their preferred chronic dialysis treatment, though approximately 20% of patients who drop out transfer to hemodialysis annually.[1] Although peritonitis remains the major reason for transfer to hemodialysis, other factors such as exit site infections, catheter-related problems, abdominal wall and inguinal hernias, loss of ultrafiltrations, and poor clearance contribute to CPD technique failure.[2] In order to permit the continuation of long-term therapy with CPD, these complications should be resolved. Routine laboratory evaluation or physical examination can detect some CPD-related problems; however, some patients require more complicated investigations to evaluate their problems properly.

Aim: To evaluate the role of 99mTc-hexakis-2-methoxyisobutylisonitrile (99mTc-MIBI) for detecting bone marrow metastases in childhood solid tumours, including lymphomas., Methods: Twenty-six children (18 males, eight females) were studied. They all had proven malignant solid tumours [Hodgkin's lymphoma (5), non-Hodgkin's lymphoma (3), neuroblastoma (9), Ewing's sarcoma (3), Langerhans cell histiocytosis (4), rhabdomyosarcoma (1) and germ cell tumour (1)] with suspected bone marrow metastases. All patients underwent computed tomography and/or magnetic resonance imaging, 99mTc-MIBI and Tc-methylene diphosphonate bone scans and bone marrow aspiration and/or biopsy. The scintigraphic evaluation of 99mTc-MIBI scans was performed according to the visual assessment of the extent and intensity of uptake. The scintigraphic score, which is the sum of the extent and intensity of uptake, was calculated for each patient. Scores of more than 2 were considered to be positive for bone marrow involvement., Results: Twenty-seven 99mTc-MIBI scans were studied for 26 patients. Twenty-two 99mTc-MIBI scans were accepted as normal bone marrow. Bone scans were also normal in these patients. Five of the 27 99mTc-MIBI scans had scores of more than 2. Bone marrow cytology revealed bone marrow metastases in these patients., Conclusion: Abnormal 99mTc-MIBI uptake correlated extremely well with bone marrow aspiration/biopsy cytology results. Non-invasive 99mTc-MIBI imaging in children with malignant solid tumours appears to be promising for the evaluation of bone marrow metastases.

Aim: The aim of this study was to evaluate the efficacy of 90Y and 186Re radiosynovectomy in patients with hemophilic synovitis., Methods: Radiosynovectomy was performed in 32 joints of 20 patients with hemophilic synovitis by using 90Y citrate colloid in the knee and 186Re sulfide colloid in the elbow, shoulder, and ankle. The indication for radiosynovectomy was the continuous presence of intra-articular blood or effusion and three or more hemorrhages into the same joint within the last 6 months. Response to therapy was first assessed at the 4th month with blood-pool imaging. Patients were followed up by clinical evaluation based on assessments of joint-bleeding frequency, using range of motion measurements at 6-month intervals for an average of 1 year (range, 9-15 months)., Results: A marked decrease (an 80%-100% decrease) in bleeding episodes was seen in 24 of 32 (75%) joints, a moderate decrease (51%-79% decrease) in 1 (9%) joint, and a mild decrease (30%-50%) in 3 (13%) joints. Frequency of intra-articular bleeding after treatment was unchanged in only 13% of the joints. The number of hemarthroses significantly decreased after therapy (p < 0.05). The mean bleeding frequency of the joints were 1.7 +/- 0.9 and 0.3 +/- 0.7 per month before and after therapy, respectively. The ratios of joints which had marked improvement after therapy were 86% in the ankle, 73% in the elbow, and 58% in the knee. There was no significant difference between percent joint range of motion limitations measured before and after therapy (p > 0.05). The correlation between therapeutic outcome (in terms of joint bleeding) and the difference of pre- and posttherapeutic blood-pool indices were significant (r = 0.594; p < 0.05), while the correlation between therapeutic outcome and pretherapeutic radiologic scale and pretherapeutic blood-pool indices were not significant (r = 0.095; p > 0.05; r = -0.089; p > 0.05, respectively)., Conclusion: Radiosynovectomy is a simple but quite effective and efficient procedure in limiting the frequency of joint hemorrhage in patients with hemophilia. Blood-pool imaging may be an objective means for monitoring therapy response in these patients.

Upper gastrointestinal system bleeding should be first taken into account when a patient complains of melena. On rare occasions, gastrointestinal bleeding may be due to primary or metastatic tumors of the gastrointestinal system. Here, we present a case in which the localization of bleeding was demonstrated by Tc-99m red blood cell labeled scintigraphy with the final diagnosis of metastases of non-small cell lung cancer to the small bowel.

Unlabelled: The aim of this study was to evaluate the value of Tc-99m ethylenedicysteine exercise renography in patients with hypertension who were suspicious for renal artery stenosis and compare the results with captopril renography., Methods: Twenty-nine patients with hypertension who were highly suspect for having renal artery stenosis were included in the study. Basal captopril exercise renograms were performed within 1 week in all patients with Tc-99m EC. Exercise was performed with a bicycle ergometer as described in the literature. Interpretations of renograms were made according to the consensus report on angiotensin converting enzyme (ACE) inhibitor renography as high, low, or intermediate probability. The sensitivity, specificity, and positive and negative predictive values were also obtained on both the kidneys and a patient basis. The results were compared with renal angiography in 19 patients., Results: Twenty of 29 patients had concordant results with both captopril and exercise studies. All discordant tests were normal with captopril but positive with exercise renograms. Nineteen patients with 38 kidneys were included for statistical data analysis. Nine kidneys, which were either atrophic or abnormal during the basal study, were excluded from the kidney-based analysis. With contrast angiography, 8 patients had renal artery stenosis. When evaluated on a kidney basis, the sensitivity, specificity, and positive and negative predictive values for captopril scans were 87%, 100%, 100%, and 95%, and for the exercise scans were 100%, 90%, 80%, and 100% respectively. When calculated on a patient basis with regard to the captopril scans, the sensitivity, specificity, and positive and negative predictive values were 87%, 81%, 77%, and 88%, and for exercise scans were 100%, 54%, 61%, and 100% respectively. There were 2 false-positive exercise scans that were found to be the result of dilated minor collecting systems and changes in kidney contours as a result of motion during exercise., Conclusions: These results indicate that exercise renography has a similar sensitivity but lower specificity compared with captopril scintigraphy in the diagnosis of renal artery stenosis. Because of the lower specificity, its use in the screening of renovascular hypertension may be limited. Exercise renography may be more useful as an investigative tool for essential hypertension.