Young boy with a long history of splenomegaly and cytopenia

A 15-year-old boy was admitted with a history of cytopenia (white blood cell count 3.170/μm, platelets 90.000/μm) associated with splenomegaly, found during investigations for recurrent mild jaundice due to Gilbert's syndrome.He was in good general health, without systemic symptoms; therefore, the leading causes of asymptomatic splenomegaly were excluded. Coagulation, liver tests and abdomen ultrasound (US) were normal, showing a hepatopetal portal flow to the colour-Doppler. There was no sign of haemolysis on haematology investigations. The C reactive protein, immune globulins levels and erythrocyte sedimentation rate were normal, excluding both an infective and an immune regulation disorder. We excluded the haematological malignancy and lymphoproliferative disorders through a peripheral blood smear and a bone marrow biopsy.His history was remarkable for neonatal sepsis, which required umbilical venous catheter during hospitalisation in a neonatal intensive care unit (NICU). The patient follow-up was interrupted for a while, probably due to his good health condition.At age 17 years, the child accessed our emergency department. for a minor trauma to the limbs, and his physical examination was unremarkable, except for the splenomegaly. We repeated the abdomen US, with colour flow Doppler (figure 1).edpract;archdischild-2019-318626v1/BLKF1F1BLK_F1Figure 1B-mode shows 1.4 cm of maximum calibre of portal vein at hilus with slightly perihilar hyperechogenicity; colour and power Doppler US shows preserved hepatopetal flow and PSV of 41 cm/s (normal range 20-40 cm/s). PSV, peak systolic velocity; US, ultrasound. QUESTIONS: What is the most likely diagnosis?Portal vein obstructionGaucher diseaseAutoimmune sclerosing cholangitisLeukemic hepatic infiltrationWhat is the gold standard imaging for diagnosis?Abdomen Doppler USContrast-enhanced CTCT without contrastUltrasound-based elastographyHow should this child be managed?Upper gastrointestinal endoscopyBeta-blocker therapyLeft-mesenteric portal vein bypass (Meso-Rex bypass)Transjugular intrahepatic porto-systemic shunt Answers can be found on page 02

An adolescent with acute abdominal pain and bowel wall thickening

A 15-year-old girl was admitted with acute crampy abdominal pain and repeated vomiting over the preceding 2\u2005hours; no fever, diarrhoea or abdominal trauma was reported. She had started oestrogen-progestin contraception 3\u2005months ago. She had sought medical advice twice in the previous weeks for self-limiting episodes of right hand swelling, without urticaria. On examination, she was unwell and in pain, with severe tenderness in the right lower quadrant, without guarding or rebound tenderness. Bowel sounds were diminished. Blood tests were unremarkable. Two hours after admission, an abdominal ultrasound scanning showed an impressive wall thickening (>1\u2005cm) of the terminal ileum, caecum and ascending colon (figure 1). Abundant free intraperitoneal fluids in the pelvis and in the hepatorenal recess were present. Figure 1Marked caecal wall thickening evidenced at the ultrasound scanning. QUESTIONS: Which of the following is the most likely diagnosis in this patient? Ileocolic intussusceptionGastrointestinal manifestation of Henoch-Sch\uf6nlein purpuraAbdominal attack of hereditary angioedema (HAE)Acute pancreatitisWhich of the following blood tests may help to confirm the diagnosis? Erythrocyte sedimentation rateC4Serum amylase: 36\u2005IU/LC1-inhibitorHow should this patient be evaluated and treated

Isolation of the left innominate artery: a question of connection

N.A

Girl With Chest Pain

N/

Splenomegaly as presentation of a wandering spleen

N/

Real-Time Tele-Mentored Low Cost "Point-of-Care US" in the Hands of Paediatricians in the Emergency Department: Diagnostic Accuracy Compared to Expert Radiologists.

The use of point-of-care ultrasonography (POC US) in paediatrics is increasing. This study investigated the diagnostic accuracy of POC US in children accessing the emergency department (ED) when performed by paediatricians under the remote guidance of radiologists (TELE POC).Children aged 0 to 18 years accessing the ED of a third level research hospital with eight possible clinical scenarios and without emergency/severity signs at the triage underwent three subsequent US tests: by a paediatrician guided remotely by a radiologist (TELE POC); by the same radiologist (UNBLIND RAD); by an independent blinded radiologist (BLIND RAD). Tele-radiology was implemented using low cost "commercial off-the-shelf" (COTS) equipment and open-source software. Data were prospectively collected on predefined templates.Fifty-two children were enrolled, for a total of 170 ultrasound findings. Sensitivity, specificity, positive and negative predictive values of TELE POC were: 93.8, 99.7, 96.8, 99.4 when compared to UNBLIND RAD and 88.2, 99.7, 96.8, 98.7 when compared to BLIND RAD. The inter-observers agreement between the paediatricians and either the unblind or blind radiologist was excellent (k = 0.93). The mean duration of TELE POC was 6.3 minutes (95% CI 4.1 to 8.5). Technical difficulties occurred in two (3.8%) cases. Quality of the transmission was rated as fair, good, very good and excellent in 7.7%, 15.4%, 42.3% and 34.6% of cases respectively, while in no case was it rated as poor.POC US performed by paediatricians in ED guided via tele-radiology by an expert radiologist (TELE POC) produced reliable and timely diagnoses. Findings of this study, especially for the rarer conditions under evaluation, need further confirmation. Future research should investigate the overall benefits and the cost savings of using tele-ultrasound to perform US "at children's bedsides", under remote guidance of expert radiologists

Characteristic of enrolled children.

<p>Characteristic of enrolled children.</p

Technical specifications for the Tele-US equipment and costs sustained.

<p>Technical specifications for the Tele-US equipment and costs sustained.</p

Components of the TELE-POC.

<p>Components of the TELE-POC.</p

START Study flow diagram.

<p>START Study flow diagram.</p