Inherited thrombocytopenias represents a heterogenous group of diseases characterized by a congenital reduction in the platelet count that could lead to a bleeding tendency. MYH9-related disorders ...are characterized by large platelets and congenital thrombocytopenia. Thrombopoietin-receptor agonists: eltrombopag and romiplostim are currently approved in many countries for the treatment of different forms of acquired thrombocytopenia, such as immune thrombocytopenia. We report, instead, the successful use of eltrombopag to treat inherited thrombocytopenia in a patient with an MHY9-related disease. This is the first report of a chronic use of eltrombopag to elevate platelets in MYH9-related disorders without side effects.
Renal artery stenosis (RAS) accounts for approximately 5%-10% of secondary renovascular hypertension in the pediatric population. It can occur as an isolated entity, or as a hypoplasia combined ...itself with stenosis. Hypoplasia, or long-segment developmental narrowing, is a rare cause of renovascular hypertension. Hyponatremic hypertensive syndrome (HHS) is a malignant complication of unilateral RAS and/or renal artery hypoplasia. Hyponatremia, hypokalemic hypochloremic metabolic alkalosis, nephrotic range proteinuria, polyuria, polydipsia, and weight loss are the most common findings. In particular, hypertension remains refractory despite aggressive antihypertensive therapy. Laboratory findings of elevated plasma levels of renin in most case suggest that the stimulation of renin release from the ischemic kidney plays an important pathophysiologic role. HHS is a diagnostic and therapeutic challenge in children. We report a case of a unilateral right renal artery hypoplasia, complicated by a segmental narrowing, in a 17-month-old male, clinically symptomatic for hypertension. We emphasize the role of ultrasound, computed tomography, and digital subtraction angiography that should be planned as reliable and non-invasive multimodal imaging approach.
Contrast-enhanced ultrasound scan (CEUS) is the application of ultrasound contrast agents (UCAs) to traditional ultrasound. Our aim is to report the use of CEUS for a prompt assessment of a suspected ...secondary splenic lymphoma in a child, which, in our experience, has allowed an accurate description of the parenchymal perfusion and vascularization pattern, leading to a confident diagnosis. We suggest that CEUS will replace Magnetic resonance imaging (MRI) or Computed tomography (CT) as standard imaging option for differential diagnosis of spleen lesions in pediatric population. As a result this will lead to decreasing the overall use of ionizing radiation and reducing the time interval to a certain diagnosis.
Neonatal breast enlargement is a hormone-related condition, mostly asymptomatic\physiological, with a well-recognizable sonographic appearance but limited data in the literature. It can be uni-or ...bilateral. Typically described in the first week of life, the transient lesion disappears spontaneously within 6 months. The main differential diagnosis is neonatal mastitis, a breast tissue infection that requires a specific antibiotic therapy. Knowledge of clinical and imaging findings is crucial, and ultrasound represents a reliable tool which allows a quick identification and an excellent examination of neonatal breast abnormalities. We aim to highlight sonographic classic features of benign neonatal breast enlargement showing how ultrasound rules out real breast masses helping to differentiate between it and mastitis when clinical and laboratory data are inconclusive. We describe a 2-day-old male with bilateral breast swelling more pronounced on the right side.
Midgut volvulus with malrotation is typical in newborns. We present our experience to emphasize the importance of suspecting midgut volvulus as a cause of abdominal pain also beyond infancy, ...particularly in relation to malrotation, and the relevance of ultrasonographic (US) signs in its diagnosis.
A total of 34 patients (10 boys, 24 girls; ages between 1 day and 12 years) diagnosed as having malrotation or malrotation with volvulus, surgically confirmed, between 2006 and 2013 were retrospectively selected among all of the patients referred to our institution for acute abdomen and bilious vomiting. All of them underwent US and color Doppler examination before surgery. The US examinations were performed with 6 to 10 MHz microconvex and 7.5 to 10 Mhz linear transducer. The transducer was placed under xiphoid, and, with axial projection, the presence of anatomic position reversed between superior mesenteric vein (SMV) and superior mesenteric artery (SMA), as a sign of malrotation, and the presence of "whirlpool sign" (WS) (wrapping of the SMV and the mesentery around the SMA), as a sign of midgut volvulus, were evaluated.
In 27 of these 34 patients, midgut volvulus was present; 7 patients had intestinal malrotation. In 2 of 7 (28%) patients with malrotation, SMA and SMV were inverted. Among the patients with volvulus, 2 showed reversed vessel position and 22 patients presented the WS in association with SMA/SMV inversion (22/27, 81%).
Midgut volvulus with malrotation can appear beyond the neonatal age group as demonstrated in our case. The WS sign is sufficiently sensitive for its diagnosis and should be routinely researched at all ages of pediatric population. Anatomic inversion between SMV and SMA seems to be not enough sensitive in isolated malrotation diagnosis.
Background
Fatigue is an important clinical and psychological aspect for a significant number of children affected by immune thrombocytopenia (ITP). To date, few studies have explored fatigue and its ...relationship with chronic ITP in pediatric age. The aim of the present multicentric pilot study is to determine fatigue perception in a large group of children with chronic ITP and their caregivers using the PedsQL Multidimensional Fatigue Scale (PedsQL MFS), and to compare the results with those of healthy control subjects.
Procedure
Children with chronic ITP aged 5‐18 years and/or caregivers of children aged 2‐18 years were enrolled. Child/adolescent self‐report was used for patients aged 5‐18 years, and parent proxy‐report for patients aged 2‐18 years. The questionnaire was offered as online survey. PedsQL MFS is composed of 18 items covering three dimensions: General Fatigue Scale, Sleep/Rest Fatigue Scale, and Cognitive Fatigue Scale.
Results
One hundred ninety‐one patients affected by chronic ITP and 248 caregivers answered the PedsQL MFS. We have highlighted that lower values of PedsQL MFS scores are obtained in the 13‐18 age group. Moreover, sleep/rest fatigue domain appears to be more compromised in all age groups. For all PedsQL MFS scores, pediatric patients with chronic ITP and their caregivers reported statistically significant worse fatigue than healthy children.
Conclusions
This study suggests that fatigue is relevant among children and adolescents affected by chronic ITP. The PedsQL MFS represents an adequate instrument for measuring fatigue in patients with chronic ITP. Therefore, symptoms of fatigue should be routinely assessed in clinical practice.
Purpose
Intussusception is one of the most common causes of paediatric emergency. Fluoroscopy-guided hydrostatic reduction is a common nonoperative management strategy for the treatment of ...intussusception. The role of pharmacological premedication in increasing the success rate of hydrostatic reduction is still controversial. The purpose of this study was to verify the presence of a possible correlation between pharmacological premedication and the percentage of hydrostatic reduction of intussusception in paediatric patients.
Materials and methods
This study considered children with a diagnosis of idiopathic intussusception treated at our hospital between January 2007 and June 2013. One group of patients underwent hydrostatic reduction by barium enema without any preliminary therapy. A second group of patients received pharmacological premedication with both a sedative and an anti-oedematous agent before the procedure.
Results
A total of 398 patients were treated with barium enema for therapeutic purposes. In the group of patients who received no premedication (
n
= 254), 165 (65 %) children achieved hydrostatic reduction of the intussusception. Among the patients who received pharmacological premedication prior to barium enema (
n
= 144), 122 (85 %) children achieved resolution of the intussusception.
Conclusions
Our study shows that the use of pharmacological premedication is effective for the reduction of the intussusception, as its limit patient stress, fluoroscopic time and radiation dose.
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