Severe bronchiolitis is uncommon in previously healthy term infants <12 months of age and when present develops soon after disease onset. Severity is predicted by young age and RSV carriage, whereas epidemiologic variables seem less likely to intervene.
The aim of this study is to evaluate the diagnostic accuracy of vertebral fractures assessment (VFA) in comparison with conventional radiography in identifying vertebral fractures in children and adolescents affected by OI. On 58 patients (33 males, 25 females; age range 1-18 years; 41 children and 17 adolescents) with osteogenesis imperfecta (OI type I, n = 44, OI type III, n = 4; OI type IV, n = 10), lateral spine images by radiographs and by dual-energy X-ray absorptiometry (DXA) were acquired. For vertebral fracture diagnosis, plain radiographs were used as "gold standard" and VFA and morphometric X-ray absorptiometry (MXA) were performed. The visualized vertebrae were 738 (97.9%) by radiographs and 685 (90.9%) by DXA of a total of 754 vertebrae from T4 to L4. VFA and MXA identified, respectively, 129 (74%) and 116 (66%) of the 175 vertebral fractures detected by radiographs. Radiographs identified 36 patients with vertebral fractures, VFA 35 and MXA 41 (6 false positives). On a per vertebra basis, radiographs and VFA had elevated agreement (93.9%; k score 0.81, 95% CI 0.76-0.86), that resulted slightly lower for MXA (90.6%; k score 0.72, 95% CI 0.65-0.78). VFA and MXA demonstrated high sensitivity (95.6 and 94.1 %, respectively) while specificity was 100% for VFA and 90.6% for MXA on a per patient basis; the agreement was excellent for VFA (98.3%; k score 0.96, 95% CI 0.89-1.03) and good for MXA (87.9%; k score 0.73, 95% CI 0.55-0.91). The diagnostic performance parameters resulted better for VFA (sensitivity 95.6%; specificity 100%; PPV 100%; NPV 97.2%), than for MXA (sensitivity 94.1%; specificity 85.4%; PPV 72.7%; NPV 97.2%). The results of our study demonstrate the reliability of VFA for diagnosis of vertebral fractures in children with OI suggesting its use as a more safe and practical alternative to conventional radiography.
Background: Cutis tricolor is a skin abnormality consisting in a combination of congenital hyper-and hypopigmented skin lesions (in the form of paired macules, patches or streaks) in close proximity to each other in a background of normal skin. It is currently regarded as a twin-spotting (mosaic) phenomenon and today is clear that not all cases of cutis tricolor represent one single entity. This phenomenon has been reported so far either: (I) as an purely cutaneous trait; (II) as a part of a complex malformation phenotype (Ruggieri-Happle syndrome, RHS) including distinct facial features, eye (cataract), skeletal (skull and vertebral defects, and long bones dysplasia), nervous system (corpus callosum, cerebellar and white matter anomalies, cavum vergae and holoprosencephaly) and systemic abnormalities; (III) as a distinct type with multiple, disseminated smaller skin macules (cutis tricolor parvimaculata); and (IV) in association with other skin disturbances [e.g., cutis marmorata telangectasica congenita (phacomatosis achromico-melano-marmorata)] or in the context of other skin (e.g., ataxia-telangiectasia and phacomatosis pigmentovascularis, PPV) or complex malformation phenotypes (e.g., microcephaly and dwarfism).Methods: (I) Review of the existing literature; and (II) information on our personal experience (clinical, laboratory and imaging data) on new cases with cutis tricolor seen and followed-up at our institutions during years 2010-2016. Results:The existing literature revealed 19 previous studies (35 cases) with pure cutaneous or syndromic cutis tricolor phenomena. Our personal experience included 5 unpublished patients (3 boys; 2 girls; currently aged 2 to 14 years) seen and followed-up at our Institutions in Italy who had: (I) skin manifestations of the cutis tricolor type (N=5); (II) skeletal abnormalities including small skull (n=2), obtuse angle of mandible (n=3), mild to moderate scoliosis (n=3), vertebral defects (n=3), and long bones bowing (n=3); mild psychomotor delay (n=3); epilepsy (n=2); anomalies of the corpus callosum (n=3); and cavum vergae (n =2).Conclusions: This study further confirms and expands the overall phenotype of cutis tricolor. By literature review and personal experience we conclude that the skin abnormalities of the cutis tricolor type are stable over time; the skeletal defects are mild to moderate and do not progress or cause relevant orthopaedic complications; the neurological/behavioural phenotype does not progress and the paroxysmal events (when present) tend to decrease over time; there is a typical facial phenotype in some patients (long, elongated face, thick and brushy eyebrows, hypertelorism, deep nasal bridge with large bulbous nose and anteverted
The tracheal isolation of Ureaplasma urealyticum from critically ill infants was investigated to determine if the organism was associated with an inflammatory response. Twenty nine neonates consecutively admitted for acute respiratory disease, with birthweights of <1301 g and no evidence of viral, chlamydial, or bacterial infections, were identified. Culture results for ureaplasmas were correlated with white celil counts and clinical and radiographic features. Sixteen infants had tracheal aspirates and/or blood specimens positive for U urealyticum. Pneumonia was diagnosed more frequently in the U urealyticum positive infants than in the 13 who were negative for the organism. The mean total white cell count, absolute neutrophil, and band form counts were significantly higher in the U urealyticum positive group than in the negative group.These data suggest that U urealyticum can induce an inflammatory response in selected individuals who present with clinical, radiographic, and, in some instances, histological features of pneumonia. (Arch Dis Child 1995; 73: F37-F40) Keywords: Ureaplasma pneumonia, white cell counts, preterm infants.During the past decade there has been increased interest in Ureaplasma urealyticum and its possible role in perinatal morbidity and mortality, including sepsis, meningitis, and pneumonia. 14 Therefore, given the pathogenic potential of this organism, the ability to target this population for a marker of severity of inflammation in order to direct treatment against U urealyticum would be welcome.U urealyticum colonisation of the nasopharynx and/or trachea of preterm neonates in the absence of other microbial pathogens is associated with an increased white cell count, indicative of a true inflammatory process.5During the course of an ongoing prospective study on the incidence and outcome of U urealyticum infection in neonates, we discovered three neonates with U urealyticum infection of the respiratory tract who had a substantial increase in white cell count.6 These observations needed to be confirmed and extended, and it was with this aim that we reviewed all neonates with acute respiratory distress recently admitted to the neonatal intensive care unit who had had specimens cultured for mycoplasmas, and we correlated culture results with white cell counts, clinical and chest radiograph findings, paying particular attention to pneumonic changes. MethodsAll infants with acute respiratory distress admitted consecutively to the neonatal intensive care unit at the University of Rome, Italy, from October 1993 to September 1994 (n=55) were included in the study. Data on maternal and perinatal risk factors (rupture of membranes, maternal fever, perinatal asphyxia and Apgar scores) were obtained by chart abstraction. The gestational age of all infants was determined using the Dubowitz score.Samples were obtained for cultures of U urealyticum within the first day of life, during the third day of life, and subsequently in some cases if the patient's respiratory condition deteriorated. T...
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