Normal Findings Research Articles

SAT437 Immunotherapy Induced Pituitary Apoplexy

Abstract Disclosure: A. Uddin: None. M.J. Gardner: None. A. Akofu: None. N. Sheung: None. S. Dejhansathit: None. N. Akabogu: None. F. Marium: None. We present a case of a 42 year old male with past history of metastatic melanoma s/p 3 cycles of ipilimumab and nivolumab immunotherapy that originally was referred to endocrinology due to elevated prolactin of 40 and MRI finding of questionable 6 mm pituitary adenoma. There was not a clear other cause of prolactin elevation. Other notable labs in pituitary hormonal evaluation were elevated FSH of 24 and LH 16 with normal testosterone 663. TSH of 4.3 and FT4 of 1.22. After the 4th cycle of ipilimumab and nivolumab he had a severe headache that was unremitting, he called his oncology nurse line which instructed him to hydrate. He presented for admission to the hospital after coming confused, unsteady on his feet and his headache had already improved. Labs demonstrated severe hyponatremia at 111 with serum osm of 238 and urine osmolality of 504 and specific gravity of 1.012. Brain MRI demonstrated a 1.2 cm pituitary lesion compared to less than 1 month prior, consistent with hypophysitis. Repeat pituitary labs showed hypogonadotropic hypogonadism with total testosterone of 39 and inappropriately normal FSH of 7 and LH of 2, central hypothyroidism with TSH 0.232, FT4 0.70, FT3 1.8, secondary adrenal insufficiency with cosyntropin stimulation test up to cortisol of 16 for partial response and ACTH of 17. Hyponatremia was managed inpatient and he was discharged on appropriate hormone replacement. Subsequent brain MRI with normal pituitary finding without enhancing lesions. Repeat hormonal testing showed persistent hormonal deficiencies with undetectable cortisol and ACTH despite holding hydrocortisone therapies. Prolactin was also tested in the 4-10 range on repeat testing. He continued on nivolumab monotherapy with oncology. This case report details a clear case of immunotherapy treatment leading to destruction of prolactinoma and presentation of pituitary apoplexy given the short time line of 1 month and cycle of therapy between radiographic and hormonal assessment. We hope this case helps bring awareness to the evolving list of conditions to survey for with immunotherapy and suggests a role of identifying prolactinomas biochemically and or radiographically to affect management. It may even suggest a role in treatment for medically refractory pituitary adenomas. Presentation: Saturday, June 17, 2023

Key Findings on Computed Tomography of the Head that Predict Death or the Need for Neurosurgical Intervention From Traumatic Brain Injury.

Traumatic brain injury (TBI) requires rapid management to avoid secondary injury or death. This study evaluated if a simple schema for quickly interpreting CT head (CTH) imaging by trauma surgeons and trainees could be validated to predict need for neurosurgical intervention (NSI) or death from TBI within 24 hours. We retrospectively reviewed TBI patients presenting to our trauma center in 2020 with blunt mechanism and GCS ≤ 12. Primary independent variables were presence of 7 normal findings on CTH (CSF at foramen magnum, open fourth ventricle, CSF around quadrigeminal plate, CSF around cerebral peduncles, absence of midline shift, visible sulci/gyri, and gray-white differentiation). Trauma surgeons and trainees separately evaluated each patient's CTH, scoring findings as normal or abnormal. Primary outcome was NSI/death in 24hours. Our population consisted of 444 patients; 21.4% received NSI or died within 24hours. By trainees' interpretation, 5.8% of patients without abnormal findings had NSI/death vs 52.0% of patients with ≥1 abnormality; attending interpretation was 8.7% and 54.9%, respectively (P < .001). Sulci/gyri effacement, midline shift, and cerebral peduncle effacement maximized sensitivity and specificity for predicting NSI/death. Considering pooled results, when ≥1 of those 3 findings was abnormal, sensitivity was 77.89%, specificity was 80.80%, positive predictive value was 52.48%, and negative predictive value was 93.07%. Any single abnormality in this schema significantly predicted a large increase in NSI/death in 24hours in TBI patients, and three particular findings were most predictive. This schema may help predict need for intervention and expedite management of moderate/severe TBI.

Autoimmune hepatitis and myositis as presenting manifestation of mixed connective tissue disease – A case report

In the course of mixed connective tissue disease (MCTD), hepatitis is very rare. Most commonly liver steatosis or elevated liver enzymes are reported and only a few cases of MCTD associated with autoimmune hepatitis were described previously. We report a case of 30-year-old female, who initially presented with autoimmune hepatitis and was treated with oral Prednisolone. Few weeks later, she presented with lower motor neuron weakness of all four limbs with normal reflexes and sensory findings. Clinically, the pattern of weakness was suggestive of a myopathic process which was confirmed with a high creatine phosphokinase levels and electromyography. She had history of Raynaud’s phenomenon with minimal skin thickening involving the extremities and face. Thus, a diagnosis of overlap syndrome/MCTD was kept. MCTD has been confirmed on high titers positive anti U-1 ribonucleoprotein antibodies. Autoimmune hepatitis and myositis as the presenting manifestation are very rare. Here, we highlight a patient of MCTD with initial symptoms of autoimmune hepatitis and myositis.

A Giant Calcified Amorphous Tumor in the Left Ventricle Presentingwith Dyspnea

Introduction: Calcified amorphous tumor (CAT) is a non-neoplastic cardiac tumor, frequently presenting as a a mass with variable sizes. Scare cases of this phenomenon have been reported so far, so its natural history and therapeutic options are still unclear. In fact, CAT diagnosing relies on pathological findings where a definite diagnosis is not reachable until the mass is removed. So, CAT is a retrospectively diagnosed following surgical intervention. Case Presentation Case Presentation: A 72-year-old man was referred for evaluation due to complaints of dyspnea. Initial coronary examinations yielded normal findings. In echocardiography, a 56 × 34 mm mass was seen in the posterior left ventricle wall, along with severe mitral stenosis and moderate to severe mitral regurgitation. The patient subsequently underwent cardiac surgery, during which the mass was completely removed through the left atrium and mitral valve. Then the patient underwent biological heart valve replacement. Post-surgery echocardiography demonstrated the absence of any residual mass, and no complications were reported during the six months follow-up period. Conclusions: The symptoms and complications of CAT during imaging manifest as a calcified mass. Due to the diagnostic reliance on pathological evidence, surgical treatment is the preferred option for excluding any malignancy and resolve and prevent further complications. Follow-up with echocardiography is advised for monitoring.

DIAGNOSTIC IMAGING AND OROTRACHEAL INTUBATION IN CLINICALLY HEALTHY WILD SUNDA PANGOLINS (MANIS JAVANICA) IN SINGAPORE.

Pangolins are amongst the most overexploited species in the world and all eight species of pangolins are threatened with extinction. These animals are rare in zoological collections and often suffer high mortality rates in captivity. Maintaining healthy populations in captivity has become more important with the declining populations in the wild, but knowledge of veterinary care of these animals is limited. Interpreting radiography and ultrasonography images in a patient can be challenging without knowledge of normal findings. The Wildlife Healthcare and Research Center (WHRC) at Mandai Wildlife Reserve (MWR) admits an average of 25 Sunda pangolins (Manis javanica) annually, which is a Critically Endangered species native to Singapore. All the pangolins are triaged on admission and anesthetized for a health assessment before release into the wild. Endotracheal intubation using an otoscope and stylet is a novel technique in the species that has been developed and is commonly performed with these pangolins. A retrospective study was done on 20 clinically healthy wild pangolins to determine normal ultrasonography and radiography findings in the species. Notable findings include the presence of radio-opaque particles in the stomachs of all pangolins, the presence of free fluid cranial to the left kidney (13/20), a spleen with multifocal hypoechoic regions (6/20), and open epiphyseal plates of long bones even in large individuals weighing as heavy as 6.8 kg. Ultrasonographic images and measurements of kidney, spleen, and adrenal gland sizes as well as intestinal, gallbladder, and urinary bladder wall thickness were also described. These diagnostic imaging findings can advance the veterinary care of captive and wild pangolins.

Development and evaluation of a point-of-care ocular ultrasound curriculum for medical students - a proof-of-concept study

BackgroundPoint-of-care Ocular Ultrasound (POCOUS) has gained importance in emergency medicine and intensive care in recent years. This work aimed to establish and evaluate a dedicated ultrasound education program for learning POCOUS-specific skills during medical studies at a university hospital.MethodsThe blended learning-based program (6 teaching units) based on recent scientific publications and recommendations was developed for students in the clinical part of their medical studies. Experts and trainers consisted of physicians from the Ear-Nose-Throat, radiology, ophthalmology and neurology specialties as well as university educational specialists. Lecture notes containing digital video links for preparation was produced as teaching material. In total, 33 students participated in the study. The education program, including the teaching materials, motivation and subjective gain in competency, was evaluated with the aid of a questionnaire (7-point Likert response format). Objective learning success was assessed on the basis of pre- and post-tests. These covered the skill areas: “anatomical basics”, “ultrasound basics”, “understanding of cross-sectional images”, “normal findings” and “pathology recognition”.ResultsIn the objective assessment of image interpretation, the participants improved significantly (p < 0.001) from pre- to post-test with a large effect size (Cohen’s d = 1.78, effect size r = 0.66). The evaluations revealed a high level of satisfaction with the course concept, teaching materials and the tutors. In addition, a high level of motivation was recorded in relation to continuing to study “ultrasound diagnostics” and “ophthalmologic diseases”. A significant (p < 0.01) positive gain was also achieved in terms of the subjective assessment of competency. This covers areas such as expertise, sonographic anatomy and performing a POCOUS examination as well as recognizing retinal detachment, globe perforation and increased optic nerve sheath diameter.ConclusionThe results of this feasibility study show that medical students accept and support a POCOUS-specific education program and are able to develop a higher objective and subjective level of competency. Future transfer to other sites and larger groups of participants seems feasible.

A Retrospective Analysis of Venographic Images of a Central Venous Port without Blood Return and Its Usable Period.

Objective In routine practice, central venous ports without blood return (CVPWBRs) are common. However, very few studies have reported on the viable period of CVPWBR use. We therefore investigated this period by retrospectively analyzing the venographic images of CVPWBRs. Methods We examined patients' venography through the CVPs at the point when they became CVPWBRs for the first time and analyzed the reasons for becoming CVPWBRs. For patients with minor complications of CVPs or normal venographic findings, we used the Kaplan-Meier method to evaluate the period for which such CVPWBRs could be used. Patients or Materials Eighty-four patients with malignancy whose CVPs became CVPWBRs for the first time between July 31, 2015, and March 12, 2020, were included. Results Nine (10.7%) patients had major complications that made the CVPs unusable. Thirty-three (39.3%) patients had minor complications, and the remaining 42 (50.0%) had normal venographic findings. For the 75 patients with minor complications or normal venographic findings who continued to use their CVPWBRs, the Kaplan-Meier method estimated that 25% of complications that might make it unusable would occur within 1,273 days. Conclusion There are two learning points in our study. First, venography is needed when the CVP becomes a CVPWBR for the first time due to the high risk, and second, CVPWBRs can be used for a relatively long period in patients without major complications. It is necessary to develop an appropriate follow-up management method for CVPWBRs in prospective studies.

Late first-trimester ultrasound findings can alter management after high-risk NIPT result.

To evaluate the impact of detailed late first-trimester ultrasound (LFTU) on the positive predictive value (PPV) of a high-risk non-invasive prenatal test (NIPT) result for various chromosomal abnormalities. This was a retrospective study of all cases undergoing invasive prenatal testing from three tertiary providers of obstetric ultrasound over 4 years, each using NIPT as a first-line screening test. Data were collected from pre-NIPT ultrasound, NIPT, LFTU, placental serology and later ultrasound examinations. Prenatal testing for chromosomal abnormalities was performed by microarray, initially using array comparative genomic hybridization and then single nucleotide polymorphism (SNP) array for the last 2 years. Uniparental disomy testing was performed by SNP array during all 4 years. The majority of NIPT tests were analyzed using the Illumina platform, initially confined to the assessment of the common autosomal trisomies, sex chromosome aneuploidies and rare autosomal trisomies (RAT), then extending to genome-wide analysis for the last 2 years. Amniocentesis or chorionic villus sampling (CVS) was performed on 2657 patients, 1352 (51%) of whom had undergone prior NIPT, with 612 (45%) of these returning a high-risk result and meeting the inclusion criteria for the study. LFTU findings significantly affected the PPV of the NIPT result for trisomies 13 (T13), 18 (T18) and 21 (T21), monosomy X (MX) and RAT but not for the other sex chromosomal abnormalities or segmental imbalances (> 7 Mb). Abnormal LFTU increased the PPV close to 100% for T13, T18, T21, MX and RAT. The magnitude of the change in PPV was highest for the most severe chromosomal abnormalities. When LFTU was normal, the incidence of confined placental mosaicism (CPM) was highest in those with a high-risk NIPT result for T13, followed by T18 and T21. After normal LFTU, the PPV for T21, T18, T13 and MX decreased to 68%, 57%, 5% and 25%, respectively. LFTU after a high-risk NIPT result can alter the PPV for many chromosomal abnormalities, assisting counseling regarding invasive prenatal testing and pregnancy management. The high PPVs of NIPT for T21 and T18 are not sufficiently modified by normal LFTU findings to alter management. These at-risk patients should be offered CVS for earlier diagnosis, particularly given the low rate of CPM associated with these aneuploidies. Patients with a high-risk NIPT result for T13 and normal LFTU findings often wait for amniocentesis or avoid invasive testing altogether given the low PPV and higher rate of CPM in this context. © 2023 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

Clinical significance of risk-reducing salpingo-oophorectomy in patients with BRCA1/2 mutation.

Serous tubal intraepithelial carcinoma (STIC) is a precursor lesion which is located in the distal fallopian tube and causes high grade serous ovarian carcinoma (HGSOC). The incidence of STIC for women underwent risk reducing salpingo-oophorectomy for BRCA mutation varies from 0.6 to 7 % and its clinical outcomes are still unclear. The aim of this study was to demonstrate the incidence of STIC and HGSOC in BRCA1/2 mutation carriers after risk reducing salpingo-oophorectomy (RRSO) and the clinical outcomes of these patients. We retrospectively reviewed the records of 48 BRCA1 and/or 2 mutation carriers who underwent prophylactic salpingo-oophorectomy with or without hysterectomy at the Department of Obstetrics and Gynecology, Bursa Uludag University between January 2000 and January 2022. BRCA 1 and/or 2 mutation carriers diagnosed by genetic testing, asymptomatic patients with no abnormal findings on pelvic examination. patients with no abnormal findings on pelvic examination and a presence of a personal history of ovarian, fallopian tube or peritoneal cancer. A total of 48 BRCA 1 and/or 2 mutation carriers underwent RRSO. STIC was diagnosed in 1 (2,0 %) patient and restaging surgery was not performed. Primary peritoneal carcinoma (PPC) did not develop during the 20 months follow-up period. One (2.0 %) patient was diagnosed with occult ovarian cancer. Restaging surgery was performed and chemotherapy treatments were given after surgery. A pelvic recurrence developed 25 months after the occult cancer diagnosis in the follow up period. One (2.0%) patient with normal histopathological findings after RRSO was diagnosed with peritoneal cancer 57 months after the operation. The risk of PPC continues after RRSO. Therefore, close follow-up procedure is very important for early diagnosis and effective treatment of patients with PPC after RRSO.

S1754 The Efficacy and Yield of Routine Duodenal Biopsy for the Diagnostic Evaluation of Celiac Disease in Patients With Normal Endoscopic Findings

S1754 The Efficacy and Yield of Routine Duodenal Biopsy for the Diagnostic Evaluation of Celiac Disease in Patients With Normal Endoscopic Findings

Does in vitro fertilization affect the hearing levels of women?

This study aimed to determine whether there is a relationship between changes in blood estrogen levels and hearing threshold levels in women undergoing in vitro fertilization (IVF). Sixty patients with normal otoscopic examination findings and scheduled for IVF treatment were included in the study. All patients underwent pure tone audiometry, tympanogram tests, and otoacoustic emission measurements (TEOAE) during IVF treatment, and estrogen levels were measured simultaneously. The patients were divided into three groups based on estrogen levels during IVF. The patients' audiological test results at the beginning and end of treatment (the 12th day) were compared between the groups. There was an increase in hearing levels on the 12th day of treatment compared to the beginning of treatment in all the study groups. This increase was significant between Group 1 and Group 2 and between Group 1 and Group 3 (p<0.05). The best hearing in audiometry was determined in the patients in Group 2. While a significant increase was observed in TEOAE (otoacoustic emission) measurements in Groups 1 and 2, a significant decrease was determined in Group 3 (p<0.05). Middle ear pressure and compliance measurements on the tympanogram tests were significantly lower in Group 3 compared to Groups 1 and 2 (p<0.05). During IVF treatment, as the estrogen level increases, it causes an increase in the level of hearing, but this increase does not continue after specific doses.

Distinct phenotypes in a cohort of anti-CASPR2 associated neurological syndromes

IntroductionAnti-contactin-associated protein-like 2 (CASPR2) is classically associated with limbic encephalitis (LE), Morvan syndrome and peripheral nerve hyperexcitability (PNH). Additional clinical features have been previously recognized. ObjectiveTo describe a cohort of patients with anti-CASPR2-associated neurological syndromes from a tertiary referral centre. MethodsRetrospective analysis of patients with positive serum anti-CASPR2 antibodies in the period between 2014 and 2021. ResultsNineteen patients were identified, 11 (57.9%) male, with a median age at symptom onset of 49.0 (31.3–63.0) years and a median time to diagnosis of 1.0 (0.0–1.8) years. The most common clinical syndromes were LE (7 cases, 36.8%), Morvan syndrome (4, 21.1%) and PNH (2, 10.5%). Six patients presented with atypical phenotypes (31.6%), comprising dysautonomia (orthostatic hypotension and Adie’s Pupil), motor tics/stereotypies, obsessive-compulsive disorder, and brainstem involvement. The most common presenting symptoms were seizures (31.6%), PNH (21.1%) and cognitive dysfunction (15.8%). One LE patient had a disease duration of 2,5 years and was initially diagnosed with dementia. CSF was normal in most cases. Brain MRI showed temporal lobe hyperintensities in 4 LE cases (57.1%). All PNH cases had myokymic discharges of fasciculations in the electromyography. Two patients had associated thymoma and 1 had lung adenocarcinoma. Eight patients (42.1%) received treatment during the acute phase and 26.3% maintenance treatment. Approximately half of the treated cases improved or stabilised, with 4 (21.1%) deaths in the whole cohort. ConclusionAnti-CASPR2-associated neurological disorders may present with isolated atypical phenotypes, a slowly progressive clinical course, and with normal CSF or imaging findings.

Motorcycle trauma unveiling massive pyeloureteral junction syndrome

This medical case study presents a rare and intriguing incidentally discovered junctional syndrome in a 36-year-old male involved in a motorcycle accident. A subsequent CT scan revealed a massive pyelocalyceal dilatation measuring 22 cm with a 12 cm-diameter pyelon, compressing the renal parenchyma (Fig1,Fig2). Remarkably, despite the extensive dilation, the patient's renal function remained unaffected, as indicated by normal laboratory findings. Prompt management involved right-side JJ stenting (Fig3,Fig4) and renal scintigraphy, which revealed that the right kidney contributed only 20% of the total renal function. This exceptional presentation highlights the significance of early detection and intervention in such cases to optimize renal health and prevent potential complications. Long-term monitoring is essential to ensure the well-being of the patient and manage this rare medical condition effectively.

Non-contrast dual-energy CT using X-map for acute ischemic stroke: region-specific comparison with simulated 120-kVp CT and diffusion-weighted MR images

PurposeX-map is a non-contrast dual-energy CT (DECT) application to identify acute ischemic stroke (AIS). Our aim was to verify region-specific characteristics of early ischemic changes (EIC) on X-map compared with simulated 120-kVp mixed-CT image and DWI.MethodsFifty AIS patients who underwent DECT and DWI were enrolled (mean age, 76 years; 34 men, 16 women). All datasets including mixed-CT image, X-map, and DWI were transformed into a standard brain atlas with 11 × 2 ROIs based on the ASPECTS + W system. ROIs with EIC on DWI, mixed-CT image, and X-map were defined as DWI-positive, mixed-CT-positive, and X-map-positive, and those with normal finding were DWI-negative, mixed-CT-negative, and X-map-negative respectively, in visual assessment by two neuroradiologists in consensus.ResultsEIC on X-maps were visually relevant to those on the other images: of 221 ROIs with mixed-CT-positive and X-map-positive, 198 (89.6%) were DWI-positive. X-map revealed moderate diagnostic accuracy for AIS compared with DWI in ROC curve analysis (AUC = 0.732). X-map identified EIC in deep white matter more sensitively than mixed-CT image: of 15 ROIs with mixed-CT-negative and X-map-positive in W segments, 14 (93.3%) were DWI-positive. X-map often showed EIC in cortical regions that were not detected on the other images: of 67 ROIs with mixed-CT-negative and X-map-positive in I and M1-M6 segments, 47 (70.1%) were DWI-negative.ConclusionsX-map is useful to detect EIC, especially in deep white matter, and may also provide additional information in acute ischemic lesions where DWI cannot be detected.

Transcranial Magnetic Stimulation Measures, Pyramidal Score on Expanded Disability Status Scale and Magnetic Resonance Imaging of Corticospinal Tract in Multiple Sclerosis.

Probing the cortic ospinal tract integrity by transcranial magnetic stimulation (TMS) could help to understand the neurophysiological correlations of multiple sclerosis (MS) symptoms. Therefore, the study objective was, first, to investigate TMS measures (resting motor threshold-RMT, motor evoked potential (MEP) latency, and amplitude) of corticospinal tract integrity in people with relapsing-remitting MS (pwMS). Then, the study examined the conformity of TMS measures with clinical disease-related (Expanded Disability Status Scale-EDSS) and magnetic resonance imaging (MRI) results (lesion count) in pwMS. The e-field navigated TMS, MRI, and EDSS data were collected in 23 pwMS and compared to non-clinical samples. The results show that pwMS differed from non-clinical samples in MEP latency for upper and lower extremity muscles. Also, pwMS with altered MEP latency (prolonged or absent MEP response) had higher EDSS, general and pyramidal, functional scores than pwMS with normal MEP latency finding. Furthermore, the RMT intensity for lower extremity muscles was predictive of EDSS functional pyramidal scores. TMS/MEP latency findings classified pwMS as the same as EDSS functional pyramidal scores in 70-83% of cases and were similar to the MRI results, corresponding to EDSS functional pyramidal scores in 57-65% of cases. PwMS with altered MEP latency differed from pwMS with normal MEP latency in the total number of lesions in the brain corticospinal and cervical corticospinal tract. The study provides preliminary results on the correspondence of MRI and TMS corticospinal tract evaluation results with EDSS functional pyramidal score results in MS.

Case Series of Rare Head and Neck Plasmacytoma: Experiences in Indonesia

Background: Solitary plasmacytoma (SP) is a rare case of plasma cell neoplasms, which only represent 5-10% of all plasma cell neoplasms that afflict two or three people per every 100,000 of the general population. This neoplasm is divided into solitary plasmacytoma of the bone (SBP) and solitary extramedullary plasmacytoma (SEP), depending on the location. Due to the rarity, SBP can be misdiagnosed as multiple myeloma (MM) while SEP as plasma cell granuloma, whereas the treatments are very different. This study aimed to present our experiences in diagnosing SP in the head and neck to differentiate them from MM and plasma cell granuloma.&#x0D; Case presentation: One case of multiple solitary plasmacytomas of the bone (SPB) in the frontal bone and one case of intraoral solitary extramedullary plasmacytoma (SEP) were presented. The SBP showed abnormality in the laboratory and the CT showed a punch-out lesion which is similar to MM. The SEP showed normal laboratory findings, but the histopathology mimicked plasma cell granuloma. Both immunohistochemistry was positive for CD138, and the SEP diagnosis was confirmed by Ki67 and κ-light chain. Complete examination from history taking, physical examination, radiology and laboratory findings, bone marrow morphology, and histopathology including immunohistochemistry examination, are very important to determine the correct diagnosis.&#x0D; Conclusion: the case was diagnosed as head and neck solitary plasmacytoma.

Nutcracker syndrome in pediatrics: initial findings and long-term follow-up results.

Nutcracker syndrome (NCS) describes a set of symptoms and signs resulting from compression of the left renal vein (LRV). There is a lack of knowledge about its natural course, diagnosis, and management, especially in children. Herein, we present our single-center experience with a large number of patients who have long-term follow-up results. All patients with NCS diagnosed between January 2011 and March 2021 were included and their data were obtained retrospectively. A total of 123 NCS patients (85 females) were included. The median age at the time of diagnosis was 12 (IQR 10-14) years, and BMI percentiles were below 5% in 38% of the cases. At the time of diagnosis, two-thirds of the patients were asymptomatic. The most common laboratory finding was nephritic proteinuria (98%), followed by microscopic hematuria (16%). Signs of LRV compression were significantly more evident in upright position Doppler ultrasonography (DUS) examination. All patients have been followed conservatively; hematuria and/or proteinuria resolved in 43 of the 108 patients (40%) within 35.8 ± 25.8months of follow-up. Control DUS was performed in 52 patients after a mean period of 39.1 ± 21.3months. The median peak velocity and diameter ratios of the LRV in the upright position were found to be decreased significantly when compared to the initial assessment (p < 0.05). Normal DUS findings were noted in 13 patients at the final evaluation. In unexplained proteinuria and/or hematuria, NCS should be considered, especially in asthenic adolescents. Our results support conservative management in children as the first-line treatment approach.

Structural ultrasound of joints and tendons in healthy children: development of normative data

BackgroundMusculoskeletal ultrasound is a well accessible technique to assess disease activity in children with juvenile idiopathic arthritis. Knowledge of reference values of joint structures is indispensable to differentiate between physiological and pathological finding. The aim of this study was to assess the structural sonographic features of joints and tendons in healthy children from several age groups (0.2–18 year), and develop a set of normative data.MethodsGreyscale ultrasound was performed in 500 healthy children (age 0.2–18 years) according to a predefined scanning protocol (Additional file 1) including the shoulder, elbow, wrist, second metacarpophalangeal joint, hip, knee, ankle, and first metatarsophalangeal joint). Demographic data and values of cartilage thickness, tendon diameters, and the degree of capsular distention measured by bone-capsular distance (BCD) were collected. Differences according to the sex were assessed by unpaired t-test. Single and multiple regression analyses were performed between the ultrasound outcomes and covariates such as age, height, weight and body mass index. Growth charts and tables were developed with respect to age. Nonparametric quantile regression was applied using the R-packages quantreg and quantregGrowth.ResultsA total of 195 male and 305 female volunteers were included between the age of 0 and 18 years (mean age 8.9; range: 0.2–17.9 years). Cartilage diminished markedly as children aged, and cartilage of the boys was significantly thicker compared to the girls in all joints (p < 0.001). In addition, cartilage became thinner as children’s height and weight increased (beta regression coefficients between − 0.27 and − 0.01, p < 0.0001). Capsular distention (i.e., BCD > 0 mm) was uncommon in the ankle, wrist and MCP2 (resp. in 3, 6, and 3% of cases). It was more common in the suprapatellar and parapatellar knee, MTP1 and posterior recess of the elbow (resp. in 34, 32, 46, and 39% of cases). In the hip, some capsular distention was always present. Age was found to be the best predictor for BCD (beta regression coefficients between 0.05 and 0.13, p < 0.0001). Height was, in addition to age, a good predictor of tendon diameter (beta regression coefficients between 0.03 and 0.14, p < 0.0001). Growth curves and tables for each variable were developed.ConclusionsReference values of sonographic cartilage thickness, BCD and diameters of tendons at several joints were established from 500 healthy children, aged between 0.2 and 18 years. Growth charts and tables were developed to distinguish normal findings from pathology in children with complaints suspicious of arthritis.

Long-Term Clinical Outcome of Abdomino-Thoracic Lymphatic Interventions of Traumatic and Non-Traumatic Lymphatic Leakage in Adults.

The aim of this study was to retrospectively evaluate the long-term results of lymphatic interventions in adults with abdomino-thoracic lymphatic pathologies. Management of abdomino-thoracic chylous effusions in adults undergoing X-ray-lymphangiography with or without lymph-vessel embolization (LVE) from 2010-2018 was reviewed. Patients underwent lymphangiography alone when imaging showed normal findings or lymphatic obstruction without leakage or reflux; otherwise, LVE was performed (leakage, reflux, obstruction with leakage or reflux, lymphatic masses). Technical and clinical success, complications, and long-term outcomes were assessed. 78 patients (47 male, median age 56.3 years) were treated for chylous effusions (60.3% traumatic, 39.7% non-traumatic). Lymphangiography showed leakage (48.7%), reflux (14.1%), obstruction (28.2%), lymphatic masses (5.1%), and normal findings (3.8%). Embolization was performed in 49/78 (62.8%) cases. Overall, treatment was clinically successful in 74.4% (mean follow-up of 28 months), with significant differences between LVE and lymphangiography (91.8% vs. 44.8%; p < 0.001), traumatic and non-traumatic etiologies (89.4% vs. 51.6%; p < 0.001), and leakage locations (p = 0.003). The clinical success of LVE did not differ between leakage etiologies or locations. Complications occurred in 5 patients (2/5 needed treatment). Patients survived significantly longer after successful treatment (2679 vs. 927 days; p = 0.044) and without malignancy (3214 vs. 1550 days; p = 0.043). Lymphatic interventions are safe and effective. LVE should be attempted whenever feasible, as success is high (>90%). Successful intervention has a positive effect on patient survival.

What Mothers Know about Newborn Bloodspot Screening and the Sources They Use to Acquire This Knowledge: A Pilot Study in Flanders.

To learn what mothers know about newborn bloodspot screening (NBS), the procedure, and the sources used, a pilot study was performed. An online questionnaire was developed, with the first part focused on characteristics and the NBS procedure, and the second on knowledge, information sources, and health care providers (HCPs). This questionnaire was accessible until 200 answers were received. The characteristics of respondents were representative for the population. Mothers gave verbal consent in 69.5% of cases, 12.5% did not, and 18% stated that no consent was requested. The 'knowledge' part contained 12 closed questions, five multiple-choice questions on sources, and assessments (5-point Likert scores) of the information transfer. The mean knowledge level was 7.2/12. Screening concepts (consequences, likelihood, sensitivity, carrier) and absence of notification of normal findings were well known. The fact that NBS is not compulsory was poorly known, and post-analysis sample handling procedures were poorly understood. Key HCPs were midwifes (80.5%) and nurses (38.5%). When the leaflet (44%) was provided, the majority read it. Mean Likert scores were 3.36, 3.38, 3.11 and 3.35 for clarity, timing appropriateness, sufficiency, and usefulness. The knowledge level and consent practices were reasonably good. Key HCP were midwives and nurses, the leaflets were supporting. This should enable a quality improvement program to a sustainable NBS program in Flanders.