Abstract
Background
SAPHO (synovitis, acne, pustolosis, hyperostosis and osteitis) syndrome is a rare autoinflammatory chronic disorder, presenting with non-infectious osteitis, sterile joint ...inflammation and skin manifestations including palmoplantar pustolosis and severe acne.
It could be often misdiagnosed for its heterogeneous clinical presentation. Treatment is challenging and, due to the rarity of this syndrome, no randomized controlled clinical trials have been conducted. Empirical treatments, including non-steroidal anti-inflammatory drugs (NSAIDs), corticosteroids, antibiotics and bisphosphonates and disease-modifying anti-rheumatic drugs (DMARDs) could be quite effective. Anti-tumor necrosis factor-alpha (anti-TNF-α) agents and interleukin-1 (IL-1) antagonists have shown promising results in refractory patients. Isotretinoin, commonly used for severe acne, has been rarely described as possible trigger of osteo-articular manifestations, in particular sacroiliitis.
Case presentation
The case of a boy, affected by acne fulminans and depression, who presented with sacroiliitis after a 10-week treatment with isotretinoin is presented. After SAPHO diagnosis, NSAIDs therapy was started but the onset of bilateral gluteal hidradenitis suppurativa required the switch to a TNF-α antagonist (Adalimumab) with the achievement of a good control of the disease. Despite specific therapy with sertraline, the patient continued to complains severe depression.
Conclusions
Our case reports a temporal association between the onset of osteo-articular symptoms and the introduction of isotretinoin, as previously described. However, this timeline is not sufficient to establish a causal role of this drug into the pathogenesis of sacroiliitis. At this regard, further studies are required. The occurrence of hidradenitis suppurativa during SAPHO course supported the introduction of TNF-α blockers with a favourable result, as reported in a few cases in literature. The association between SAPHO syndrome and depressive mood disorders is already reported. Our patient experienced severe depression whose trend seems to be independent from the course of the main disease. Currently, it is not clarified if depression could be considered reactive to the underling disease or if it forms an integral part of the autoinflammatory disorder.
Citomegalovirus (CMV) infects approximately 1% of live newborns. About 10% of the infants affected by congenital CMV infection are symptomatic at birth and up to 60% of these infants will develop ...permanent neurological disabilities. Depending on gestational age (GA) at the time of infection, the involvement of central nervous system (CNS) can lead to malformations of cortical development, calcifications, periventricular white matter lesions and cysts, ventriculomegaly and cerebellar hypoplasia.
We report the MRI findings in a Caucasian female born at 32 weeks of post-menstrual age with post-birth diagnosis of congenital CMV infection showing an unusual and peculiar marked T2 hyperintensity of the inner part of olfactory bulbs in addition to the CMV related diffuse brain involvement. Despite the known extensively described fetal and neonatal Magnetic Resonance Imaging (MRI) findings in CMV infected fetuses and newborns, any in vivo MRI depiction of olfactory system damage have never been reported so far. Nevertheless, in murine studies CMV is known to infect the placenta during pregnancy showing particular tropism for neural stem cells of the olfactory system and previous neuropathologic study on CMV infected human fetal brains from 23 to 28 weeks of GA reported damage in the olfactory bulbs (OB) consisting in disseminated cytomegalic cells, inflammation, necrosis and neuronal and radial glial cell loss. Therefore, we assume an OB involvement and damage in congenital CMV infection.
To our knowledge this is the first in vivo MRI evidence of OB damage in a newborn with congenital CMV infection that may give new insights on CMV infection.
Low platelet count might promote resistance to pharmacological closure with indomethacin and ibuprofen of a hemodynamically significant patent ductus arteriosus (hsPDA). However, no studies have ...investigated if this occurs with paracetamol.
We retrospectively assessed the correlation between platelet count, mean platelet volume (MPV), and plateletcrit (PCT), as well as the effectiveness of paracetamol in closing hsPDA in infants born at 23
-31
weeks of gestation who were treated with 15 mg/kg/6 h of i.v. paracetamol for 3 days.
We studied 79 infants: 37 (47%) Had closure after a course of paracetamol and 42 (53%) did not. Platelet count and PCT did not correlate with paracetamol success or failure in closing hsPDA, while MPV was lower at birth (10.7 ± 1.4 vs. 9.5 ± 1.1;
< 0.001) and prior to starting therapy (11.7 ± 1.9 vs. 11.0 ± 1.6;
= 0.079) in refractory infants. Regression analysis confirmed that the low MVP measured prior to starting the treatment increased the risk of hsPDA paracetamol closure failure (OR 1.664, 95% CI 1.153-2.401).
The greater MPV correlated positively with the effectiveness of paracetamol in closing hsPDA, while platelet count and PCT did not influence closure rates. Additional studies are needed to confirm our results.
Sarcoidosis in pediatric age is uncommon and challenging diagnosis, because manifestations can be significantly variable and non-specific since it is a multisystem disease, and virtually any organ ...system may be involved.
In this report, we describe the case of a 12-year-old girl presenting with fatigue and weight loss, with a painless hepato-splenomegaly without additional clinical signs on physical examination. In our patient, once we had ruled out infections, malignancies and granulomatous diseases of childhood, we made diagnosis of sarcoidosis, finding suggestive histological features in two different tissues (liver and lymph nodes) with lung involvement.
Our case points out that pediatricians should consider sarcoidosis in the differential diagnosis in case of systemic symptoms, even in absence of other specific clinical clues, because they represent the most common clinical manifestations on presentation in children, in order to refer promptly the young patient to specialist evaluation.
Objectives
Our aim in this study was to assess the effect of the Predictive Intelligent Control of Oxygenation (PRICO®) system on cerebral (rSO2C) and splanchnic (rSO2S) oxygenation in a cohort of ...preterm infants with frequent desaturations.
Methods
Twenty infants with gestational age <32 weeks (n = 20) were assigned in random sequence to 12 h of automated or manual adjustment of FiO2. Over this period, they were studied continuously by near‐infrared spectroscopy (NIRS).
Results
We found that rSO2C 68.0% (60.5%–74.7%) vs. 68.5% (62%–72%); p = .824 and rSO2S 27.0% (17.3%–45.7%) vs. 27.0% (15%–53%); p = .878 were similar during automatic and manual control of FiO2. Time spent with SpO2 90%–95% was higher during the automatic than manual control of FiO2, while time spent with SpO2 <80% or >95% was lower.
Conclusions
Automated control of FiO2 with PRICO® system did not improve brain and splanchnic oxygenation in comparison with manual control in a cohort of preterm infants, but it significantly decreased SpO2 fluctuations and limited the duration of both hypoxemia and hyperoxemia.
Introduction
It has recently been reported that it is possible to monitor lung oxygenation (rSO2L) by near‐infrared spectroscopy (NIRS) in preterm infants with respiratory distress syndrome (RDS). ...Thus, our aim was to assess the possibility of monitoring rSO2L in infants with evolving and established bronchopulmonary dysplasia (BPD) and to evaluate if rSO2L correlates with BPD severity and other oxygenation indices.
Methods
We studied 40 preterm infants with gestational age ≤30 weeks at risk for BPD. Patients were continuously studied for 2 h by NIRS at 28 ± 7 days of life and 36 weeks ± 7 days of postmenstrual age.
Results
rSO2L was similar at the first and second NIRS recordings (71.8 ± 7.2 vs. 71.4 ± 4.2%) in the overall population, but it was higher in infants with mild than in those with moderate‐to‐severe BPD at both the first (73.3 ± 3.1 vs. 71.2 ± 3.2%, p = .042) and second (72.3 ± 2.8 vs. 70.5 ± 2.8, p = .049) NIRS recording. A rSO2L cutoff value of 71.6% in the first recording was associated with a risk for moderate‐to‐severe BPD with a sensitivity of 66% and a specificity of 60%. Linear regression analysis demonstrated a significant positive relationship between rSO2L and SpO2/FiO2 ratio (p = .013) and a/APO2 (p = .004).
Conclusions
Monitoring of rSO2L by NIRS in preterm infants with evolving and established BPD is feasible and safe. rSO2L was found to be higher in infants with mild BPD, and predicts the risk for developing moderate‐to‐severe BPD and correlates with other indices of oxygenation.
BACKGROUNDIt has been reported that preterm infants can develop feeding intolerance during phototherapy (PT) and that PT can affect mesenteric perfusion in these patients. AIMSOur aim was to assess ...if PT can decrease regional splanchnic oxygenation (rSO2S) measured by near infrared spectroscopy (NIRS). STUDY DESIGNWe prospectively studied infants with gestational age of 25-34 weeks with hyperbilirubinemia requiring PT. Splanchnic regional oxygenation (rSO2S), oxygen extraction fraction (FOES), and cerebrosplanchnic oxygenation ratio (CSOR) were recorded before, during, and after PT discontinuation. RESULTSDuring PT rSO2S and CSOR significantly decreased and this effect lasted for some hours after its interruption. FOES contemporary increased, although this effect was not statistically significant. CONCLUSIONSPT treatment decreases splanchnic oxygenation in preterm infants likely due to peripheral vasodilation which triggers a redistribution of blood flow. These results can help explain the association between PT and the development of feeding intolerance in preterm infants.
Introduction
Noninvasive markers more accurate than FiO2 would be useful to assess the severity of RDS and guide its treatment. Our aim was to assess for the first time the possibility of ...continuously monitoring lung oxygenation (rSO2L) by near‐infrared spectroscopy (NIRS) and to evaluate whether rSO2L correlates with other oxygenation indices and RDS severity.
Methods
We carried out this proof‐of‐concept study on 20 preterm infants with RDS requiring noninvasive respiratory support. Patients were continuously studied for 24 h by NIRS and rSO2L was correlated with SpO2/FiO2 ratio, a/APO2, and O.I.
Results
The overall value of rSO2L was 80.1 ± 6.2%, without significant differences between the right and left hemithorax (80.2 ± 6.7 vs. 80.0 ± 5.7%; p = 0.869). Mean values of total, right, and left rSO2L did not significantly change during the 24‐h study period. Linear regression analysis demonstrated a significant positive relationship between total rSO2L and SpO2/FiO2 ratio (p < 0.001) and a/APO2 (p = 0.040), and a negative relationship between total rSO2L and O.I. (r = −0.309; p = 0.022).
Conclusions
Continuous monitoring of rSO2L by NIRS in preterm infants with RDS is feasible and safe. The correlation of rSO2L with other indices of oxygenation and RDS severity supports the accuracy and reliability of this measurement.
Aim
To investigate the association between morphine exposure in the neonatal period and neurodevelopment at 2 and 5 years of age while controlling for potential confounders.
Method
We performed a ...retrospective, single‐centre cohort study on 106 infants (60 males, 46 females; mean gestational age 26 weeks SD 1) born extremely preterm (gestational age < 28 weeks). Morphine administration was expressed as cumulative dose (mg/kg) until term‐equivalent age. Neurodevelopmental outcome was assessed at 2 years with the Bayley Scales of Infant and Toddler Development, Third Edition, Dutch version and at 5 years with the Wechsler Preschool and Primary Scale of Intelligence, Third Edition, Dutch version. Multiple linear regression analysis was used to assess the association between morphine exposure and outcome.
Results
Sixty‐four out of 106 (60.4%) infants included in the study received morphine. Morphine exposure was not associated with poorer motor, cognitive, and language subscores of the Bayley Scales of Infant and Toddler Development, Third Edition, Dutch version at 2 years. Morphine exposure was associated with lower Full‐Scale IQ scores (p = 0.008, B = −9.3, 95% confidence interval CI = −15.6 to −3.1) and Performance IQ scores (p = 0.005, B = −17.5, 95% CI = −27.9 to −7) at 5 years of age.
Interpretation
Morphine exposure in infants born preterm is associated with poorer Full‐Scale IQ and Performance IQ at 5 years. Individualized morphine administration is advised in infants born extremely preterm.
What this paper adds
A significant association between morphine exposure and neurodevelopmental impairment at 5 years was observed.
Higher exposure to painful and stressful procedures during the neonatal period was associated with poorer abilities at 5 years of age.
Differently from previous studies on morphine, this association was also considered in the statistical analysis.
A more individualized morphine administration is advised in infants born extremely preterm to counteract the negative effects of high stress without affecting neurodevelopment.
Many very preterm infants are treated with phototherapy (PT) for hyperbilirubinemia and it has been reported that PT can negatively affect gut perfusion. Thus, our aim was to evaluate the occurrence ...of feeding intolerance in the course of PT in these patients.
We retrospectively studied infants born at 25
+0
-31
+6
weeks from November 2017 to April 2020 who required PT during the first two weeks of life. Patients were used as their own controls recording for each one the occurrence of feeding intolerance after starting PT and the resumption of feeding tolerance after its termination.
We studied 125 preterm infants of whom 58 (46%) developed a feeding intolerance which disappeared in 47 (81%) of them at the end of PT. Regression analysis showed a trend toward a not significant decrease of risk of feeding intolerance in infants with higher birth weight and age at the start of the first course of PT.
We found that about half of our patients developed a transient feeding intolerance during PT that ceased in the vast majority of them after termination of the therapy. Further studies are necessary to confirm the correlation between PT and feeding intolerance.
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