CT scans of 21 patients(intrathoracic goiter=7. Castleman disease=6, pulmonary carcinoid tumor=3, parathyroid adenoma=1, thyroid carcinoma=1, paraganglioma=1, benign pleural mesothelioma=1, sclerosing hemangioma=1) with mediastinal tumors that are known to be enhanced with intravenous injection of contrast media, were studied retrospectively to investigate the enhancing capabilities f those tumors and to describe their CT findings. The degree of enhancement was estimated by visual inspection with a grading system. All but one case of cystic parathyroid adenoma showed enhancement on post-contrast scan. The degree of enhancement was not helpful in differentiating these tumors. Characteristic location and pattern of tumor extension were found in cases of intrathoracic goiter, parathyroid adenoma, thyroid carcinoma and paraganglioma. Calcifications were found in intrathoracic goiter(5/7). Castleman disease(2/6). Pulmonary carcinoid(1/3), thyroid carcinoma. Necrotic low-attenuation areas were in intrathoracic goiter(7/7), parathyroid adenoma, thyroid carcinoma and paraganglioma. In conclusion, mediastinal tumors that are known to be enhanced in the literature were enhanced with rare exception, and if we consider the predilection site of those tumors, the scope of differential diagnosis can reasonably be narrowed.
Carcinoid Tumor ; Contrast Media ; Diagnosis, Differential ; Goiter, Substernal ; Injections, Intravenous ; Paraganglioma ; Parathyroid Neoplasms ; Retrospective Studies ; Thyroid Gland ; Thyroid Neoplasms ; Tomography, X-Ray Computed

Despite its benign nature, chronic subdural hematoma (SDH) can be fatal if surgical intervention is delayed. Here, we report on bilateral chronic SDH in an 84-year-old man who died of duret hemorrhage in the brain stem and ischemia in the occipital and temporal lobes. We discuss the necessity for urgent surgical intervention to treat bilateral chronic SDH, and provide a review of the relevant literature.

Intrahepatic stones are frequently associated with recurrent pyogenic cholangiohepatitis and complete surgical removal of the stones is almost always difficult because of the large number of stones and associated bile duct strictures. One hundred and seventy-nine patients with retained intrahepatic stones underwent percuttaneous stone removal utilizing a preshaped angulated catheter and a Dormia basket under fluoroscopy with a combination of techniques including irrigation-suction. crushing of large stones. balloon dilatation of strictures and extracorporenal shock wave lithotripsy. The procedure was performed through a mature T-tube tract (177 patients) and a mature transhepatic tube tract (two patients). Stones were exclusively intrahepatic in 130 patients. whereas 49 patients also had stones in the common bile duct. In 91 (50.8%) of 179 patients. the stones were completely removed and in 36 (20.1%) patients most of the stones were removed. The overall success rate was 70.9% ngulation deformity. stricture of bile ducts and impacted stones were the factor most often responsible for failure, No significant complications were observed. Fluoroscopicalyy-guided percutaneous interventional procedures with a preshaped angulated catheter is an useful complementary procedure to surgery for patients with intrahepatic stones. the major benefits of an individually fitted angulated catheter are its safety and easy access to small peripheral bile ducts.
Bile Ducts ; Catheters* ; Common Bile Duct ; Congenital Abnormalities ; Constriction, Pathologic ; Dilatation ; Fluoroscopy ; Humans ; Lithotripsy ; Shock

Purpose: Hematuria and proteinuria have various causes and consequential outcomes in children. Immunosuppressants are needed in some children with biopsy-proven glomerulonephropathy but have many adverse effects. Since the clinical practice patterns of Korean pediatric nephrologists are diverse, we surveyed their opinions. Methods: Using a clinical vignette, the survey was emailed to all Korean Society of Pediatric Nephrology members. The questionnaires included diagnosis, examination, medications, and dietary recommendations for patients with hematuria and proteinuria. Results: A total of 32 clinicians (5.48%, 22 pediatric certificated nephrologists) responded to the survey. Most responders (87.5%) suspected immunoglobulin A nephropathy, and 68.8% replied that kidney biopsies were a diagnostic tool. Renin-angiotensin system inhibition (62.5%) or steroids (18.8%) were selected as the treatment. Salt and protein intakes were usually encouraged as dietary reference intakes (34.4% and 65.6%, respectively). Conclusions Children with abnormal urinalysis have various causes, treatments, and prognoses. As treatments such as immunosuppressants can have many adverse effects, it is necessary to confirm an accurate diagnosis and indications of treatments before starting the treatment. Recommendations for a diet should not hinder growth.

Nephrocalcinosis often occurs in infants and is caused by excessive calcium or vitamin D supplementation, neonatal primary hyperparathyroidism, and genetic disorders. Idiopathic infantile hypercalcemia (IIH), a rare cause of nephrocalcinosis, results from genetic defects in CYP24A1 or SLC34A1. Mutations in CYP24A1, which encodes 25-hydroxyvitamin D 24-hydroxylase, disrupt active vitamin D degradation. IIH clinically manifests as failure to thrive and hypercalcemia within the first year of life and usually remits spontaneously. Herein, we present a case of IIH wih CYP24A1 mutations.An 11-month-old girl visited our hospital with incidental hypercalcemia. She showed failure to thrive, and her oral intake had decreased over time since the age of 6 months. Her initial serum parathyroid hormone level was low, 25-OH vitamin D and 1,25-OH vitamin D levels were normal, and renal ultrasonography showed bilateral nephrocalcinosis. Whole-exome sequencing revealed compound heterozygous variants in CYP24A1 (NM_000782.4:c.376C>T [p.Pro126Ser] and c.1310C>A [p.Pro437His]). Although her hypercalcemia and poor oral intake spontaneously resolved in approximately 8 months, we suggested that her nephrocalcinosis and renal function be regularly checked in consideration of potential asymptomatic renal damage. Hypercalcemia caused by IIH should be suspected in infants with severe nephrocalcinosis, especially when presenting with failure to thrive.

Atypical hemolytic uremic syndrome (aHUS) is a rare disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury without any association with preceding diarrhea. Dysregulation of the complement system is the most common cause of aHUS, and monoclonal humanized anti-C5 antibodies are now recommended as the first-line treatment for aHUS. However, if the complement pathway is not the cause of aHUS, C5 inhibitors are ineffective. In this study, we report the second reported case of aHUS caused by DGKE mutations in Republic of Korea. The patient was an 11-month-old infant who presented with prodromal diarrhea similar to typical HUS, self-remitted with conservative management unlike complement-mediated aHUS but recurred with fever. While infantile aHUS often implies genetic dysregulation of the complement system, other rare genetic causes, such as DGKE mutation, need to be considered before deciding long-term treatment with C5 inhibitors.

Background: Colorectal epithelial neoplasm extending into the submucosal gut-associated lymphoid tissue (GALT) can cause difficulties in the differential diagnosis. Regarding GALT-associated epithelial neoplasms, a few studies favor the term “GALT carcinoma” while other studies have mentioned the term “GALT-associated pseudoinvasion/epithelial misplacement (PEM)”. Methods: The clinicopathologic characteristics of 11 cases of colorectal epithelial neoplasm associated with submucosal GALT diagnosed via endoscopic submucosal dissection were studied. Results: Eight cases (72.7%) were in males. The median age was 59 years, and age ranged from 53 to 73. All cases had a submucosal tumor component more compatible with GALT-associated PEM. Eight cases (72.7%) were located in the right colon. Ten cases (90.9%) had a non-protruding endoscopic appearance. Nine cases (81.8%) showed continuity between the submucosal and surface adenomatous components. Nine cases showed (81.8%) focal defects or discontinuation of the muscularis mucosae adjacent to the submucosal GALT. No case showed hemosiderin deposits in the submucosa or desmoplastic reaction. No case showed single tumor cells or small clusters of tumor cells in the submucosal GALT. Seven cases (63.6%) showed goblet cells in the submucosa. No cases showed oncocytic columnar cells lining submucosal glands. Conclusions Our experience suggests that pathologists should be aware of the differential diagnosis of GALT-associated submucosal extension by colorectal adenomatous neoplasm. Further studies are needed to validate classification of GALT-associated epithelial neoplasms.

Posterior reversible encephalopathy syndrome (PRES) is a rare clinico-radiological syndrome that is characterized by an acute onset of headache, vomiting, visual abnormalities, confusion, and/or seizures and is typically accompanied with edema of predominantly posterior portions of the cerebral hemispheres. Here, we report a case of PRES with cerebral hemorrhage that occurred in a female pediatric patient with uncontrolled nephrotic syndrome. The patient's magnetic resonance imaging (MRI) studies showed asymmetric white matter abnormalities, cerebral hemorrhage, and restricted diffusion. After controlling her hypertension and nephrotic syndrome, her neurologic symptoms improved, and follow-up MRI findings revealed interval resolving of the white matter lesions.
Cerebral Hemorrhage* ; Cerebrum ; Child* ; Diffusion Magnetic Resonance Imaging ; Diffusion* ; Edema ; Female ; Follow-Up Studies ; Headache ; Humans ; Hypertension ; Magnetic Resonance Imaging ; Nephrotic Syndrome* ; Neurologic Manifestations ; Posterior Leukoencephalopathy Syndrome* ; Seizures ; Vomiting ; White Matter

BACKGROUND: Introduction of calcineurin inhibitor (CNI) has markedly improved the outcome of kidney transplantation. While therapeutic drug monitoring is used to adjust the dosage of CNI, some patients, particularly children, still suffer from rejection, infection, and CNI toxicity. This study was conducted in order to assess the adequacy of immunosuppression using pharmacodynamic monitoring. METHODS: Pharmacodynamic monitoring was performed for 64 pediatric kidney allograft recipients. Expression of nuclear factor of activated T lymphocytes (NFAT)-regulated genes in patients' mononuclear cells was measured by quantitative polymerase chain reaction of interleukin-2, interferon-gamma (IFN-gamma), and granulocyte-macrophage colony stimulating-factor before (trough) and 1.5 hour (peak) after ingestion of tacrolimus and the residual gene expression (RGE) was calculated. Global immune response was assessed by Cylex-ImmuKnow assay. Trough and peak levels of tacrolimus were measured and clinical findings of rejection episodes and infectious complications were reviewed retrospectively. RESULTS: Global immune response measured byImmuKnow did not show correlation with trough and peak levels of tacrolimus. Adenosine triphosphate level of ImmuKnow was higher in patients with Epstein-Barr virus (EBV) infection than in those without infectious complications (515.4+/-149.0 ng/mL vs. 342.7+/-155.3 ng/mL, P=0.006). Mean RGE of the three NFAT-regulated genes showed negative correlation with tacrolimus peak levels. RGE of IFN-gamma was lower in patients with other infections except EBV than in those without infectious complications (34.0%+/-7.5% vs. 56.0%+/-30.2%, P <0.001). CONCLUSIONS: RGE of NFAT-regulated genes and ImmuKnow did not show significant correlation with clinical manifestation of under- or over-suppression of immune function in pediatric kidney allograft recipients. Further studies are required for development of optimal pharmacodynamic monitoring for pediatric kidney transplantation recipients.
Adenosine Triphosphate ; Allografts ; Calcineurin* ; Child ; Drug Monitoring ; Eating ; Gene Expression ; Herpesvirus 4, Human ; Humans ; Immunosuppression ; Interferon-gamma ; Interleukin-2 ; Kidney ; Kidney Transplantation* ; Polymerase Chain Reaction ; Retrospective Studies ; T-Lymphocytes ; Tacrolimus

The Russell-Silver syndrome (RSS) is a disease characterized by intrauterine growth retardation with preserved head circumference, facial dysmorphism and short stature. Reported renal and urinary manifestations of RSS include horseshoe kidney, renal tubular acidosis, hydronephrosis, ureteropelvic obstruction and vesicoureteral reflux. Here we report a case of end-stage renal disease associated with RSS, which, to the best of our knowledge, has not been reported yet.
Acidosis, Renal Tubular ; Fetal Growth Retardation ; Head ; Hydronephrosis ; Kidney ; Kidney Failure, Chronic ; Silver-Russell Syndrome ; Vesico-Ureteral Reflux