Publications by authors named "Hiorns M"

Pelviureteric junction obstruction, also known as ureteropelvic junction obstruction, is a congenital narrowing of the urinary excretory tract at the junction between the renal pelvis and the ureter and is a common cause of congenital pelvicalyceal dilatation. The outcome is variable, from spontaneous resolution to renal parenchymal function loss in cases of untreated high-grade obstruction. Abnormalities in renal ascent, rotation and vascularity can be associated with pelviureteric junction obstruction and easily overlooked radiologically.

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Background Children with pediatric inflammatory syndrome temporally associated with SARS-CoV-2 (PIMS-TS), also known as multisystem inflammatory syndrome in children, present with abdominal pain among other nonspecific symptoms. Although initial imaging features of PIMS-TS have been reported, the duration of sonographic features remains unknown. Purpose To describe the abdominal US features of PIMS-TS at initial presentation and follow-up.

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Objective: Dextranomer-hyaluronic acid (Deflux(®)), the most widely used compound in the endoscopic treatment of vesico-ureteric reflux (VUR) today, is believed to provoke only minimal inflammation. Reports of calcification of Deflux(®) are increasing. We ascertain the incidence of Deflux(®) calcification appearing as distal ureteric calculi on ultrasound.

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Inactivating mutations in phosphate-regulating endopeptidase (PHEX) cause X-linked hypophosphatemic rickets (XLHR) characterized by phosphaturia, hypophosphatemia, bony deformities, and growth retardation. We assessed the efficacy of combined calcitriol and orally administered phosphate (Pi) therapy on longitudinal growth in relation to age at treatment onset in a retrospective, single-center review of children with XLHR and documented PHEX mutations. Growth was compared in those who started treatment before (G1; N = 10; six boys) and after (G2; N = 13; five boys) 1 year old.

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Persistent cloaco is the most severe type of anorectal malformation encountered in children. Patients with cloacal anomalies have a high incidence of associated anomalies most commonly: urinary tract and spinal. Persistent cloaca remains a difficult reconstructive challenge but it is now possible to anatomically correct the defect with surgery in the majority of patients.

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The co-existence of renal and mullerian anomalies is well-recognised. Multicystic dysplastic kidneys (MCDK) are known to be associated with the presence of genital cysts in both males and females, but this is the first report of a prenatally diagnosed MCDK associated with a non-communicating cystic uterine cavity. The management of these abnormalities in childhood is not well-established.

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Computed tomography (CT) and magnetic resonance imaging (MRI) are increasingly valuable tools for assessing the urinary tract in adults and children. However, their imaging capabilities, while overlapping in some respects, should be considered as complementary, as each technique offers specific advantages and disadvantages both in actual inherent qualities of the technique and in specific patients and with a specific diagnostic question. The use of CT and MRI should therefore be tailored to the patient and the clinical question.

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We report a 35-week preterm neonate with bladder agenesis and bilateral dysplastic kidneys. A suprapubic catheter was inadvertently inserted into one of the larger inferior cysts of the left dysplastic kidney. A postmortem MRI scan was performed with the findings being confirmed on autopsy.

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Imaging of the gastrointestinal (GI) tract in children continues to evolve, with new techniques, both radiological and non-radiological, being added to the repertoire. This article provides a summary of current imaging techniques of the GI tract (primarily the upper GI tract) and the relationship between those techniques. It covers the upper GI series and other contrast studies, US, CT and MRI.

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Objective: We routinely perform a cystourethroscopy 3 months after initial ablation of posterior urethral valves. The aim of this study was to determine the predictive value of the urethral appearance on preoperative micturating cystourethrogram (MCUG) for further valve resection at check cystoscopy.

Patients And Methods: We retrospectively reviewed 31 consecutive boys (aged 4-18 months) who underwent check cystoscopy and repeat MCUG between 2006 and 2008.

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Objectives: Necrotizing enterocolitis (NEC) with multiple organ dysfunction syndrome (MODS) carries significant morbidity and mortality. There is extensive experimental evidence to support investigation of therapeutic hypothermia in infants with these conditions. We aimed to establish the feasibility and safety of mild hypothermia in preterm neonates with NEC and MODS as a prelude to a randomized trial.

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An association between necrotizing enterocolitis (NEC) and poor neurodevelopmental outcome is increasingly recognized. A progression of preexisting intraventricular hemorrhage (IVH) or new IVH during an episode of NEC may be contributory. We investigated the incidence of IVH in infants with NEC.

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Background: Vascular calcification is associated with increased morbidity and mortality in stage V chronic kidney disease, yet its early pathogenesis and initiating mechanisms in vivo remain poorly understood. To address this, we quantified the calcium (Ca) load in arteries from children (10 predialysis, 24 dialysis) and correlated it with clinical, biochemical, and vascular measures.

Methods And Results: Vessel Ca load was significantly elevated in both predialysis and dialysis and was correlated with the patients' mean serum Ca x phosphate product.

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Rationale: It is unclear whether airway wall thickening and emphysema make independent contributions to airflow limitation in chronic obstructive pulmonary disease (COPD) and whether these phenotypes cluster within families.

Objectives: To determine whether airway wall thickening and emphysema (1) make independent contributions to the severity of COPD and (2) show independent aggregation in families of individuals with COPD.

Methods: Index cases with COPD and their smoking siblings underwent spirometry and were offered high-resolution computed tomography scans of the thorax to assess the severity of airway wall thickening and emphysema.

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Background: The radiological findings after laparoscopic heminephrectomy in children are not widely reported.

Objective: To document the range of imaging appearances following laparoscopic heminephrectomy in children.

Materials And Methods: We reviewed all imaging and all radiology reports performed on children in our institution who had undergone laparoscopic heminephrectomy over a 6-year period.

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Background: Vascular calcification occurs in the majority of patients with chronic kidney disease, but a subset of patients does not develop calcification despite exposure to a similar uraemic environment. Physiological inhibitors of calcification, fetuin-A, osteoprotegerin (OPG) and undercarboxylated-matrix Gla protein (uc-MGP) may play a role in preventing the development and progression of ectopic calcification, but there are scarce and conflicting data from clinical studies.

Methods: We measured fetuin-A, OPG and uc-MGP in 61 children on dialysis and studied their associations with clinical, biochemical and vascular measures.

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In addition to its classical role in calcium-phosphate homeostasis, vitamin D has anti-inflammatory effects that may influence vascular disease. This study examined the impact of vitamin D levels on the vascular phenotype in 61 children who had been on dialysis for >or=3 mo and in 40 age-matched control subjects. All dialysis patients were prescribed daily oral 1-alpha hydroxyvitamin D(3).

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Urinary hydrometrocolpos is rarely diagnosed and is most often attributed to a persistent urogenital sinus or a cloaca. We report the case of a neonate in which the use of dynamic MRI allowed accurate preoperative diagnosis of urinary hydrometrocolpos secondary to ectopic drainage of a small left pelvic kidney, associated with a bicornuate uterus.

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Cardiovascular disease is increasingly recognized as a life-limiting problem in young patients with chronic kidney disease, but there are few studies in children that describe its determinants. We studied the association of intact parathyroid hormone (iPTH) levels and their management on vascular structure and function in 85 children, ages 5-18 years, who had received dialysis for > or =6 months. Compared to controls, dialysis patients had increased carotid intima-media thickness and pulse-wave velocity.

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Central venous access is increasingly becoming the domain of the radiologist, both in terms of the insertion of central venous catheters (CVCs) and in the subsequent management of these lines. This article seeks to provide an overview of the CVC types available for paediatric patients and a more detailed explanation of the spectrum of complications that may lead to catheter malfunction. A standard catheter contrast study or 'linogram' technique is described.

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