The need for assessment of ureteric function in the patient with an obviousely dilated ureter has increased particularly with the added spectrum of asymptomatic patients presenting with hydrone-phrosis and hydroureter on antenatal and perinatal ultrasound. To assess the influence of ureteral status on kidney washout during $^{99m}Tc$-DTPA diuretic renography, ureteral images were reviewed in 80 children referred for hydronephrosis. A scintigraphically abnormal ureter was defined as an intense and continuous image of > 10 min during diuretic renography. Out of them, a total of 16 nephroureteral systems in 12 children with scintigraphically abnormal ureter were analyzed. A diuretic washout index using response half time (t1/2) by linear fitting after lasix injection, was determined on renal (Kt1/2) and ureteral (Ut1/2) curves (diuretic renogram vs. diuretic ureterogram). Diuretic ureterogram curve patterns corresponding to normal (type I), obstructive (II) and non-obstructive (III) cases were described. Compared with X-ray data, diuretic renography was highly sensitive (88%) and specific (99%) for detecting any ureteral abnormality. Despite an obstructive Kt1/2 (>20 min), no patient with an abnormal ureter underwent therapy at the ureteropelvic junction because the hydronephrosis regressed after surgery at the lower level. Our data indicate that the abnormal ureter findings during diuretic renography have to be recognized before therapy for children with hydeonephrosis.
Purpose: Most of diagnosis in the pediatric hydronephrosis patients have been performed $^{99m}Tc$-DMSA renal scan. Then the region of interest (ROI) is set for comparative analysis of uptake ratio in left-right kidney after acquiring the image. But if the equipment set an automatic ROI, the ROI could include expanded renal pelvis due to hydronephrosis and the uptake ratio of left-right kidney will be incorrect result. Therefore this study compared both ROIs including expanded renal pelvis and excluding renal pelvis through experiment using normal kidney phantom and expanded renal pelvis phantom and suggested setting method of improved ROI. In addition, this study have been helped by readout doctor for investigate distinction radiopharmaceutical uptake between renal cortex and remained urine by expanded renal pelvis. Materials and Methods: The both of renal phantoms were filled with water and shacked with $^{99m}TcO_4$ 111 MBq. In order to describe the expanded renal pelvis, the five latex balloon were all filled with 10 mL water and each of balloon was mixed with $^{99m}TcO_4$ 18.5, 37, 55.5, 74, 92.5 MBq. And we made phantom with fixed $^{99m}TcO_4$activity of 37 MBq and mixed water 5, 10, 15, 20, 25 mL in each balloon. The left kidney was fixed its shape and the right kidney was modified like as hydronephrosis kidney by attached the latex balloons. And the acquiring counts were 2 million. After acquisition, we compared the image of ROI with Expanded renal pelvis and the image of ROI without renal pelvis for analyzing difference in the uptake ratio of left-right kidney and for reproducibility, set the ROI 5 times in the same images. Patients were injected $^{99m}Tc$-DMSA 1.5~1.9 MBq/kg and scanned 3 to 4 hours after injection. The each of 3 skillful radio technologists performed the comparing estimation by setting ROI. To determine statistical significance between two data, SPSS (ver. 17) Wilcoxon Signed Ranks Test was used. Results: As a result of renal phantom's experiment, we compared with average of counts Background (BKG) ratios in the setting of ROI including expanded renal pelvis and setting of excluding expanded renal pelvis. Therefore, they can obtain changed counts and changed ratios. Patient also can obtain same results. In addition, the radiopharmaceutical uptake in expanded renal pelvis was come out the remained urine that couldn't descend to ureter by the help of readout doctor. Conclusion: As above results, the case of setting ROI including expanded renal pelvis was more abnormally increasing uptake ratio than the case of setting ROI excluding expanded renal pelvis in analysis the uptake ratio in left-right kidney of hydronephrosis. Because of the work convenience and prompted analysis, the automatic ROI is generally used. But in case of the hydronephrosis study, we should set the manual ROI without expanded renal pelvis for an accurate observation of the uptake ratio of left-right kidney since the radiopharmaceutical uptake in expanded renal pelvis is the remained urine.
Purpose: A duplicated ureter is congenital renal malformations with ureter in two. Patients with duplicated ureter are in force to $^{99m}Tc-DMSA$ scan at surgery before and after. In existing examination, at produce result after $^{99m}Tc-DMSA$ scan, didn't compare to upper pole and lower pole with malformed kidney and compared to only relative uptake ratio. Therefore, this study will examine about utility of set a partial region of interest and to functional recovery of renal cell through change of upper pole uptake ratio of malformed kidney by setting each partial region of interest in upper pole and lower pole of malformed kidney in $^{99m}Tc-DMSA$ examination in surgery before and after. Materials and Methods: Pediatric patients with malformed kidney of incomplete duplicated ureter, 15 patients were enrolled in this study. Scanning were scan 3 to 4 hours after injection of $^{99m}Tc-DMSA$ 1.5 ~ 1.9 MBq/kg. Region of interest were each set in normal kidney, upper pole and lower pole with malformed kidney. Region of interest were set with same condition and method to images of surgery before and after that radio technologist 1 person, resident of nuclear medicine 1 person and doctor of urology together. Therefore, this study were compared to uptake ratio (A: B: C) that normal kidney (A), lower pole of malformed kidney (B) and upper pole of malformed kidney (C) about uptake ratio changes of malformed kidney in follow-up examination of surgery before and after. Results: When compared to 15 patients, uptake ratios were increased 7 persons and decreased 8 persons. Among increased 7 persons, it were periods of follow-up examination that 2 persons were 14 months, 4 persons were 12 months and 1 person was 8 months after surgery. Among decreased 8 persons, it were periods of follow-up examination that 4 persons were 12 months 3 persons were 6 months and 1 persons were 4 months after surgery. Conclusion: Existing study could not see the exact uptake ratio changes of malformed kidney because using only the overall Left-Right kidney uptake ratios. But a setting partial region of interest was able to see exactly what changes in the uptake of each upper pole and lower pole of malformed kidney. Because recovery of renal parenchymal cells is difficult in an evaluation of short period of time, follow-up examination should be made in long period of time. How to set up partial region of interest be thought that it would be useful.
Right-sided heart failure is a major problem among patients with congenital heart diseases, due to the prevalence of congenital heart defects and the association of pulmonary hypertension. More attention is focused on the structure of the right heart particularly in association with congenital heart defects and chronic lung disease. The right ventricle (RV) may support the pulmonary circulation, and sometimes the systemic circulation (systemic RV) in congenital heart defects. Despite major progress being made, assessing the RV remains challenging, often requiring a multi-imaging approach and expertise (echocardiography, magnetic resonance imaging, nuclear and cineangiography). Evidence is accumulating that RV dysfunction develops in many of these patients and leads to considerable morbidity and mortality. While there is extensive literature on the pathophysiology and treatment of left heart failure, the data for right-sided heart failure is scarce. Therefore RV function in certain groups of congenital heart disease patients needs close surveillance and timely and appropriate intervention to optimise outcomes. An understanding of RV physiology and hemodynamics will lead to a better understanding of current and future treatment strategies for right heart failure. This will review right-sided heart failure with the implications of volume and pressure loading of the RV in congenital heart diseases.
We experienced two cases of Rotor syndrome in brothers who were a 13 year-old boy and an 11 year-old boy, respectively. They presented with icteric scleras for a few months. Their common laboratory characteristics were as follows: Direct bilirubin was more increased than indirect bilirubin, but aminotransferases were normal. Plasma indocyanine green (ICG) test revealed hepatic excretory defect: plasma ICG concentrations 15 minutes after intravenous injection were 80.45% and 78.28%, respectively. 99mTc-DISIDA Hepatobiliary scan showed that severely decreased hepatic extraction with mild cardiac blood pool, markedly delayed biliary excretion in both intra- & extra- hepatic bile ducts, delayed visualization of gall bladder, and markedly delayed intestinal biliary passage. Needle liver biopsy showed normal hepatic histology without pigmentation.
Mitochondria play essential role in eukaryotic cells including in the oxidative phosphorylation and generation of adenosine triphosphate via the electron-transport chain. Therefore, defects in mitochondrial DNA (mtDNA) can result in mitochondrial dysfunction which leads to various mitochondrial disorders that may present with various neurologic and non-neurologic manifestations. Mutations in the nuclear gene polymerase gamma (POLG) are associated with mtDNA depletions, and Alpers-Huttenlocher syndrome is one of the most severe manifestations of POLG mutation characterized by the clinical triad of intractable seizures, psychomotor regression, and liver failure. The hepatic manifestation usually occurs late in the disease's course, but in some references, hepatitis was reportedly the first manifestation. Liver transplantation was considered contraindicated in Alpers-Huttenlocher syndrome due to its poor prognosis. We acknowledged a patient with the first manifestation of the disease being hepatic failure who eventually underwent liver transplantation, and whose neurological outcome improved after cocktail therapy.
The Bethesda System for Reporting Thyroid Cytopathology (TBSRCT) is crucial for cytopathologists to use a standardized, category-based reporting system for thyroid fine needle aspirations and is effective for clear communication with the referring physicians. The new Bethesda System for Reporting Thyroid Cytopathology, the third edition in 2023, provides several key updates. The most important update is the assignment of only single name for each of the six diagnostic categories: (I) nondiagnostic; (II) benign; (III) atypia of undetermined significance; (IV) follicular neoplasm; (V) suspicious for malignancy; and (VI) malignant. An implied risk of malignancy (ROM) for each of six categories has been updated based on extensively published data since the second edition of TBSRTC in 2017 and offers both an average ROM for each category and the expected range of cancer risk. Estimated final ROM after excluding "Noninvasive Follicular Thyroid Neoplasm with Papillary Like Nuclear Features (NIFTP)" for each of six categories has been updated based on the reported mean decreases in the ROM if excluding NIFTP. For atypia of undetermined significance (AUS) category, the subcategorization is simplified and more formalized into 2 subgroups, AUS-nuclear atypia or AUS-other, based on the implied ROM and molecular profiling. For the pediatric thyroid disease, pediatric ROMs and management algorithms are newly added for the same six reporting categories for this age group. New or revised disease nomenclatures including high-grade follicular-derived carcinoma has been updated according to the recently published 2022 World Health Organization Classification of Thyroid Neoplasms. Brand new two chapters are added including clinical perspectives and imaging studies (Chap. 13) and the use of molecular and other ancillary tests (Chap. 14). The atlas is updated with new images to illustrate more effectively for new disease entity and diagnostic criteria.
Jo, Hee Cheol;Lee, Seong Wook;Jung, Hyun Joo;Park, Jun Eun
Clinical and Experimental Pediatrics
/
v.59
no.sup1
/
pp.92-95
/
2016
Neuroblastomas are sometimes associated with abnormal constitutional karyotypes, but the XYY karyotype has been rarely described in neuroblastomas. Here, we report a case of an esthesioneuroblastoma in a boy with a 47, XYY karyotype. A 6-year-old boy was admitted to our hospital because of nasal obstruction and palpable cervical lymph node, which he first noticed several days previously. A polypoid mass in the right nasal cavity was detected through sinuscopy. Biopsy of the right nasal polyp was performed. Based on the result, the patient was diagnosed with a high-grade esthesioneuroblastoma. Nuclear imaging revealed increased uptake in both the right posterior nasal cavity and the right cervical IB-II space, suggesting metastatic lymph nodes. Cytogenetic analysis revealed a 47, XYY karyotype. Twelve courses of concurrent chemotherapy were administered. Three years after the completion of chemotherapy, the patient had had no disease recurrence. He manifested behavioral violence and temper tantrums, so we started methylphenidate for correction of the behavior.
Kostopoulou, Eirini;Gil, Andrea Paola Rojas;Spiliotis, Bessie E.
Annals of Pediatric Endocrinology and Metabolism
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v.23
no.4
/
pp.204-209
/
2018
Purpose: Growth hormone transduction defect (GHTD) is characterized by severe short stature, impaired STAT3 (signal transducer and activator of transcription-3) phosphorylation and overexpression of the cytokine inducible SH2 containing protein (CIS) and p21/CIP1/WAF1. To investigate the role of p21/CIP1/WAF1 in the negative regulation of the growth hormone (GH)/GH receptor and Epidermal Growth Factor (EGF)/EGF Receptor pathways in GHTD. Methods: Fibroblast cultures were developed from gingival biopsies of 1 GHTD patient and 1 control. The protein expression and the cellular localization of p21/CIP1/WAF1 was studied by Western immunoblotting and immunofluorescence, respectively: at the basal state and after induction with $200-{\mu}g/L$ human GH (hGH) (GH200), either with or without siRNA CIS (siCIS); at the basal state and after inductions with $200-{\mu}g/L$ hGH (GH200), $1,000-{\mu}g/L$ hGH (GH1000) or 50-ng/mL EGF. Results: After GH200/siCIS, the protein expression and nuclear localization of p21 were reduced in the patient. After successful induction of GH signaling (control, GH200; patient, GH1000), the protein expression and nuclear localization of p21 were reduced. After induction with EGF, p21 translocated to the cytoplasm in the control, whereas in the GHTD patient it remained located in the nucleus. Conclusion: In the GHTD fibroblasts, when CIS is reduced, either after siCIS or after a higher dose of hGH (GH1000), p21's antiproliferative effect (nuclear localization) is also reduced and GH signaling is activated. There also appears to be a positive relationship between the 2 inhibitors of GH signaling, CIS and p21. Finally, in GHTD, p21 seems to participate in the regulation of both the GH and EGF/EGFR pathways, depending upon its cellular location.
Kim, Ji Eun;Lee, Eun Kyung;Lee, Jae Min;Bae, Soon Hwan;Choi, Kwang Hae;Lee, Young Hwan;Hah, Jeong Ok;Choi, Joon Hyuk;Kong, Eun Jung;Cho, Ihn Ho
Clinical and Experimental Pediatrics
/
v.57
no.5
/
pp.226-231
/
2014
Purpose: Kikuchi-Fujimoto disease (KFD) is a benign disease, which is characterized by a cervical lymphadenopathy with fever, and it often mimics malignant lymphoma (ML). 2-[$^{18}F$]fluoro-2-deoxy-D-glucose positron emission tomography/computed tomography ($^{18}F$-FDG PET/CT) is a powerful imaging modality for the diagnosis, staging and monitoring of ML, with the limitations including the nonspecific FDG uptake in infectious or inflammatory processes. This study compared clinical manifestations and PET/CT findings between KFD and ML patients. Methods: We retrospectively reviewed the medical records of 23 patients with KFD and 33 patients with ML, diagnosed histopathologically, between January 2000 and May 2013 at the Department of Pediatrics, Yeungnam University Medical Center. Among them, we analyzed the clinical manifestations, laboratory findings and characteristics, and the amount of $^{18}F$-FDG uptake between 8 KFD and 9 ML patients who had $^{18}F$-FDG PET/CT. Results: The $^{18}F$-FDG PET/CT maximum standardized uptake values ($SUV_{max}$) ranged from 8.3 to 22.5 (mean, 12.0) in KFDs, and from 5.8 to 34.3 (mean, 15.9) in MLs. There were no significant differences in $SUV_{max}$ between KFDs and MLs. $^{18}F$-FDG PET/CT with ML patients showed hot uptakes in the extranodal organs, such as bone marrow, small bowel, thymus, kidney, orbit and pleura. However, none of the KFD cases showed extranodal uptake (P<0.001). $^{18}F$-FDG PET/CT findings of KFD with nodal involvement only were indistinguishable from those of ML. Conclusion: Patients who had extranodal involvement on PET/CT were more likely to have malignancy than KFD.
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