Common Community-acquired Bacterial Skin and Soft-tissue Infections in Children: an Intersociety Consensus on Impetigo, Abscess, and Cellulitis Treatment.

PURPOSE: The main objective of this article was to offer practical suggestions, given the existing evidence, for identifying and managing bacterial impetigo, abscess, and cellulitis in ambulatory and hospital settings. METHODS: Five Italian pediatric societies appointed a core working group. In selected conditions, specially trained personnel evaluated quality assessment of treatment strategies according to the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) methodology. Only randomized controlled trials (RCTs) and observational studies were included for quality assessment according to the GRADE methodology. MEDLINE, Ovid MEDLINE, EMBASE, and Cochrane Library databases were searched with a strategy combining MeSH and free text terms. FINDINGS: The literature review included 364 articles focusing on impetigo, skin abscess, and cellulitis/orbital cellulitis. The articles included for quality assessment according to the GRADE methodology for impetigo comprised 5 RCTs and 1 observational study; for skin abscess, 10 RCTs and 3 observational studies were included; for cellulitis and erysipelas, 5 RCTs and 5 observational studies were included; and for orbital cellulitis, 8 observational studies were included. Recommendations were formulated according to 4 grades of strength for each specific topic (impetigo, skin abscesses, cellulitis, and orbital cellulitis). Where controversies arose and expert opinion was considered fundamental due to lack of evidence, agreement according to Delphi consensus recommendations was included. IMPLICATIONS: Based on a literature review and on local epidemiology, this article offers practical suggestions for use in both ambulatory and hospital settings for managing the most common bacterial SSTIs.

Chest Imaging of a rare case of cat-scratch disease in a 2-years-old baby.

Cat-scratch disease (CSD) is usually a self-limiting infection that in the majority of cases occurs as lymphadenitis in children who have been scratched or bitten by a cat. Rarely, Bartonella henselae is cause of fever of unknown origin (FUO), with dissemination to various organs, mimicking an inflammatory rather than a lymphoproliferative disease. This manuscript will present a case of thoracic manifestations of CSD in an immunocompetent 2-years baby without history of cat contact, with fever of unknown origin, investigated by chest CT and MRI.

Case Report: Percutaneous Treatment of Multiple Echinococcal Cysts Presenting as Abdominal Palpable Mass.

We report the case of an adolescent Moroccan girl with abdominal pain and palpable mass in the upper right side of the abdomen. In the emergency department, an abdominal ultrasound showed hepatomegaly and eight active liver cysts, compatible with cystic echinococcosis. Serology for Echinococcus granulosus confirmed the diagnosis. Other sites of localization were excluded. Treatment involved albendazole combined with puncture, aspiration, injection, re-aspiration, performed only for the most medial cysts. Periodical follow-up with abdominal ultrasound and with abdominal magnetic resonance imaging showed a progressive involution of all cysts. The treatment with albendazole was stopped after, overall, 6 months, and monthly ultrasound scan were planned as follow-up. In case of hepatic cysts, E. granulosus should be excluded, especially in children from endemic countries. A multidisciplinary approach with pediatric infectious disease specialists, radiologists, and surgeons is fundamental for disease management.

Quantitative Analysis of Apparent Diffusion Coefficient for Disease Assessment in Paediatric Inflammatory Bowel Disease.

OBJECTIVE: The aim of the study was to establish an apparent diffusion coefficient (ADC) cut-off value to classify active and non-active lesions in inflammatory bowel disease. METHODS: We reviewed 167 paediatric magnetic resonance enterographies executed for suspected inflammatory bowel disease by using a 1.5- and 3-T scanner. We assessed the presence and activity of the disease by using morphologic and functional parameters such as the ADC. Each patient could have more than 1 examinations. Quantitative assessment of disease activity in the ADC map was measured placing 3 regions of interest in the areas of highest inflammation and the mean value was calculated, patients without sign of inflammation were assessed at 2 standardised site. Ileocolonoscopy, esophagogastroduodenoscopy, surgery, and video-capsule endoscopy were used as standards of reference. RESULTS: We enrolled 34 patients and 35 examinations: radiological findings of disease were identified in 29 examinations and 44 lesions were detected. Six patients had negative results and ADC assessment was taken at the terminal ileum and cecum. A total of 56 bowel segments were included in the study. Image analysis revealed 39 active lesions (69.6%) and their ADC values were lower compared to the ones of non-active segments. For each scanner a cut-off value was found (sensitivity: 0.91, specificity: 0.89 for 1.5 T and 0.81 for 3 T). Inter-rater agreement on disease activity between ADC values and magnetic resonance enterography results and between ADC values and the standard of reference were very good. CONCLUSIONS: ADC can provide a scanner-based quantitative measurement of disease activity.

Optical coherence tomography morphology and evolution in cblC disease-related maculopathy in a case series of very young patients.

PURPOSE: To describe the retinal structure of a group of patients affected by methylmalonic aciduria with homocystinuria cblC type, caused by mutations in the MMACHC gene, using spectral domain optical coherence tomography (SD-OCT). METHODS: Young patients (n = 11, age 0-74 months) with cblC disease, detected by newborn screening or clinically diagnosed within 40 days of life, underwent molecular analysis and complete ophthalmic examination, including fundus photography and SD-OCT. In one case, we also performed fluorescein angiography (FA) and standard electroretinography (ERG). RESULTS: Molecular analysis of the MMACHC gene fully confirmed cblC disease in nine of 11 patients. Two patients harboured only a single heterozygous pathogenic MMACHC mutation and large unbalanced rearrangements were excluded by array-CGH analysis in both. All patients except two showed a bilateral maculopathy. In general, retinal changes were first observed before one year of age and progressed to a well-established maculopathy. Measurable visual acuities ranged from normal vision, in keeping with age, to bilateral, severe impairment of central vision. Nystagmus was present in six patients. Spectral domain optical coherence tomography (SD-OCT) showed macular thinning with severe alterations in outer, and partial sparing of inner, retinal layers. CONCLUSION: Patients affected by cblC disease may frequently show an early onset maculopathy with variable ophthalmoscopic appearance. Spectral domain optical coherence tomography (SD-OCT) broadens the knowledge of subtle retinal alterations during the disease's progression and helps to shed light on the pathological mechanism of maculopathy development.

[A rare case of intussusception in a seventeen year old boy: diagnosis, management and review of the literature.] / Un raro caso di invaginazione intestinale in un adolescente: diagnosi, management e revisione della letteratura.

Intussusception is the most common cause of intestinal obstruction and acute abdomen in the first year of life. Approximately in the 80% of cases intussusception occurs when the last ileal loop is pulled into the cecum, passing through the ileocecal valve, and finally resulting in the displacement of cecum in the upper abdominal. It could be related with mesenteric adenitis because enlarged lymph nodes, together with peristalsis, can serve as "lead point". Other forms of intussusception are ileo-ileal and colo-colic. The aetiology in infants and adults can be very variable and most often linked with secondary causes, such as benign or malignant lesions (polyps, tumors, lymphomas, intestinal duplication cyst, Meckel's diverticulum). In this paper we describe a rare case of idiopathic ileo-ileal intussusception in a 17 year old boy. We also discuss, with a careful analysis of the literature, the diagnostic and therapeutic protocol in case of intussusception ileo-colic approved at the Meyer Children's Hospital.

Metatropic dysplasia in third trimester of pregnancy and a novel causative variant in the TRPV4 gene.

Prenatal diagnosis of skeletal dysplasias is particularly difficult for many reasons and differentiating these disorders in the prenatal period can be challenging because they are rare and many of the ultrasound findings are not necessarily pathognomonic for a specific disorder. The diagnosis is often made just after birth or exitus. The prenatal diagnosis of osteochondrodysplasias is based predominantly upon fetal ultrasound findings and it focuses substantially on the possible lethality of the disorder, without always being able to find a specific name for the disorder. Metatropic dysplasia is a rare osteochondrodysplasia due to mutations in the TRPV4 gene: TRPV4 is a cation channel, non-selectively permeable to calcium, encoded by a gene on chromosome 12q24.11; it is widely expressed and involved in many different physiological processes through responses to several different stimuli (physical, chemical, and hormonal) in ciliated epithelial cells. The exact incidence of this disorder is not known, however less than a hundred cases have been reported at present, with only two prenatal reports but without any reference to the molecular test. We describe the first report of molecular diagnosis of metatropic dysplasia carried out in prenatal diagnosis: the molecular testing of the TRPV4 (transient receptor potential cation channel, subfamily V, member 4, MIM *605427) gene in our case, in fact, detected a causative variant, confirming the diagnostic suspicion, which was made possible thanks also to the utilization of MRI and CT scan. In our case different imaging methods together with the close cooperation of a multidisciplinary team and test availability, allowed an accurate diagnosis.

Scoliosis and Pectus Excavatum in Adolescents: Does the Nuss Procedure Affect the Scoliotic Curvature?

BACKGROUND: Pectus excavatum (PE) is known to be associated with adolescent idiopathic scoliosis (AIS). The correction of severe PE requires a mini-invasive procedure (MIRPE), with a metal bar positioned and left in the chest for 3 years. Adolescence seems to be the more appropriate time not only for MIRPE but also for AIS peak progression. This study was designed to answer the question whether, in adolescents, MIRPE could affect mild/moderate AIS. METHODS: We carried out a meta-analysis focused on defining the natural progression of untreated AIS. Inclusion criteria were as follows: AIS patients -age 10-18 years old -Cobb angle <40°-none treated as orthotics/electrostimulation/surgery. The expected outcome was the percentage of patients who improved, worsened, or hold steady of their condition at follow-up. Between 2008 and 2014, we followed up a cohort of 67 adolescents with severe PE treated with MIRPE, assessing whether AIS underwent a modification in the period between bar insertion and removal. RESULTS: Meta-analysis included 9 studies with 1641 AIS patients. Although heterogeneous (I(2) = 99.5%, P < .0001), the overall percentage of progression for untreated AIS was 42.5% (CI 18.2%-72.2%). In our follow-up group who underwent MIRPE, 34 out of 67 patients had concurrent AIS with a Cobb angle >10° (range 10°-45°). We demonstrated that MIRPE had a favorable effect on AIS, with a mean improvement of 1.5° (CI 0.64-2.44; P = .0011). CONCLUSION: In our PE patients with AIS, MIRPE had a beneficial effect also on the spine. From our preliminary results, it seems that MIRPE should be offered during puberty as a timely option for treating PE and stabilizing mild/moderate scoliosis progression, when concurrent.

Successful Propranolol Treatment of a Kaposiform Hemangioendothelioma Apparently Resistant to Propranolol.

A newborn with unresectable kaposiform hemangioendothelioma associated with Kasabach Merritt phenomenon, unresponsive to vincristine and prednisone, received second-line treatment with propranolol at a dose of 2 mg/kg/day, starting at 2 months of life and continued for 13 months. There was only slight reduction in tumor mass, but measurement of propranolol levels showed extremely low plasma concentrations. The propranolol dose was progressively increased to 3.5 mg/kg/day, leading to a substantial increase in plasma levels associated with clinically relevant tumor reduction. This case highlights the importance of relating propranolol dose to its plasma concentration before considering the treatment ineffective for this vascular tumor.

Impact of psychological interventions on reducing anxiety, fear and the need for sedation in children undergoing magnetic resonance imaging.

Children undergoing magnetic resonance imaging examination frequently experience anxiety and fear before and during the scanning. The aim of the present study was to assess: i) whether and to what extent psychological interventions might reduce anxiety and fear levels; ii) whether the intervention is related to a decrease in the need for sedation. The interventions consisted of three activities: a clown show, dog interaction and live music. The emotional status (anxiety and fear) of the children was evaluated before and after the activities through a rating scale questionnaire. The results showed that the activities had high effectiveness in reducing the level of anxiety and fear and decreased the need for sedation in the experimental group compared to the control group. This approach proved to be a positive patient experience, helping to alleviate children's anxiety and fear, decreasing the need for sedation, and was cost-effective.

Progressive extreme heterotopic calcification.

The formation of ectopic calcifications in soft tissues can occur either sporadically or as a genetically determined condition, and is seen only infrequently. We report on a girl in whom widespread, rapidly progressive ectopic calcifications were detected shortly after birth. Calcifications became present around all joints, tendons and ligaments, but did not involve internal organs and skin, and eventually caused almost complete immobility of the child at 2 years. There were no other health problems and cognitive development was normal. We compare the manifestations in the child to the characteristics of known entities causing ectopic calcifications and conclude the child differs to each. Laboratory evaluation failed to identify autoimmune phenomena as well as calcium metabolism or other biochemical abnormalities; molecular studies did not identify occurrence of mutations in disease genes known to be involved in ectopic calcifications. We conclude the manifestations in the child represent an unreported entity of hitherto unknown etiology.

Left coronary artery stenosis with post-stenotic aneurysm after arterial switch operation before and after coronary revascularisation surgery.

We report the case of a child with severe and atypical stenosis of the left main coronary artery, which occurred late after arterial switch operation for transposition of the great arteries. Cardiac computed tomography accurately defined the lesion, showing the presence of post-stenotic dilation, guided the surgical approach and assessed coronary patency after revascularisation surgery.

Nonossifying fibroma: A possible pitfall in F18-FD-PET/CT imaging of Hodgkin's disease.

A 14-year-old girl was examined for a right lateral neck swelling and radiographic mediastinal widening. Biopsy of a right supraclavicular lymph node demonstrated the nodular sclerosing form of Hodgkin's lymphoma. An 18F-fluorodeoxyglucose-positron emission tomography/computed tomography (F18-FDG-PET/CT) study showed several pathological areas of lymph-node uptake in the upper mediastum and right distal tibia. Radiography of the tibia revealed a nonossifying fibroma in the site corresponding to the distal tibial uptake. The PET appearance of benign fibro-osseous lesions may be similar to those of skeletal metastases. Information obtained by the CT component of the PET/CT study and by conventional radiography can be useful in preventing erroneous interpretations of F18-FDG-PET uptake.

Image diagnosis in McCune-Albright syndrome.

McCune-Albright syndrome consists of polyostotic fibrous dysplasia, precocious puberty and cafè-au-lait skin lesions. Bone lesions are characterized by the presence of fibrous connective tissue with a characteristic whorled pattern and containing trabeculae of immature non-lamellar (woven) bone. They may be solitary (monostotic) or multiple (polyostotic). Commonly involved bones include the femur, tibia, ribs and facial skeleton. In the fibula pseudo-cystic areas and 'ground glass'-like areas, in the femur 'shepherd crook' deformation due to weight on a less resistant bone and secondary to many cortical microfractures, are typical. MRI gives the exact delimitation of the lesions and is especially indicated in the followup of monostotic forms and in the outcome of surgical corrections. Bone scintigraphy with technetium 99 is essential in the follow-up of the disease. Ultrasonography is very useful in ovarian cyst follow-up and in the detection of thyroid and adrenal nodules and testicular microlithiasis.

Severe malignant osteopetrosis caused by a GL gene mutation.

Infantile malignant autosomal recessive osteopetrosis is a genetically heterogeneous disease caused by the inability of OCLs to resorb and remodel bone, resulting in generalized osteosclerosis and obliteration of marrow spaces and cranial foramina. The classical clinical features are pathological fractures, visual impairment, and bone marrow failure. Two human genes have been described as the cause of this form of osteopetrosis: the T-cell immune-regulator-1 (TCIRG1) gene, which is mutated in >50% of the patients, and the chloride channel 7 (ClCN7) gene, which accounts for approximately 10% of cases. We report the clinical, radiographic, and histopathologic findings of the first human osteopetrosis case caused by a mutation in the grey-lethal (GL) gene. The patient, a 9-day-old male infant, presented with a very severe osteopetrotic phenotype including substantial hepatosplenomegaly since birth, cytopenia, and progressive major liver failure. Skeletal radiographs revealed a generalized increase in bone density with loss of corticomedullary differentiation. Histopathologic bone examination showed the typical osteopetrotic changes, with absence of resorptive activity, and osteoclasts, slightly decreased in number, with evident morphological alterations.

Oral, facial, digital, vertebral anomalies with psychomotor delay: a mild form of OFD type Gabrielli?

A girl with oral, facial, and digital anomalies presented at birth with a large cleft palate filled by a nasopharyngeal mass and was found later to have several vertebral anomalies and mental retardation. A similar phenotype has been previously reported in a sporadic male patient [Gabrielli et al., 1994: Am J Med Genet 53:290-293], suggesting a new variant form of oral-facial-digital syndrome.