A 74-year-old transgender female presented with urinary difficulty and a painful lesion at her neo-vagina, 47 years after undergoing gender-affirming vaginoplasty. Initial biopsy reported Low-Grade ...Squamous Intraepithelial Lesion (LGSIL), while MRI revealed no inguinal or pelvic lymphadenopathy. Patient underwent partial neo-vaginectomy with wide local excision and formal urethroplasty. Pathology revealed verrucous carcinoma of the neovagina, a rare neoplasm in transgender female patients. This case highlights the importance of considering squamous cell carcinoma in the differential diagnosis of patients presenting lesions at neo-vagina and the need of screening methods for early detection of such neoplasms.
(1) Purpose: A patient with scleritis may have an associated systemic disease, which is often autoimmunological and seldom infectious in origin. The data regarding such associations in Hispanic ...populations are scarce. Therefore, we evaluated the clinical characteristics and systemic-disease associations of a cohort of Hispanic patients with scleritis. (2) Methods: A retrospective review of the medical records (January 1990-July 2021) of two private uveitis practices in Puerto Rico was performed. Clinical characteristics and systemic-disease associations observed either at presentation or diagnosed as a consequence of the initial workup were recorded. (3) Results: A total of 178 eyes of 141 patients diagnosed with scleritis were identified. An associated autoimmune disease was present in 33.3% of the patients (rheumatoid arthritis, 22.7%; Sjögren's syndrome, 3.5%; relapsing polychondritis, 2.8%; sarcoidosis, 1.4%; systemic lupus erythematosus, 1.4%; and systemic vasculitis, 0.7%). An associated infectious disease was present in 5.7% of the patients (2.13%, syphilis; 1.41%, herpes simplex; 1.14%, herpes zoster; and 0.71%, Lyme disease). One patient had all-trans retinoic-acid-associated scleritis. Statistical analysis revealed that patients with nodular anterior scleritis were less likely to have an associated immune-mediated disease (OR: 0.21;
= 0.011). (4) Conclusion: Rheumatoid arthritis was the most common systemic autoimmune disease association, while syphilis was the most common infectious disease associated with scleritis patients. Our study suggests that patients with nodular scleritis have a lower risk of having an associated immune-mediated disease.
Dexmedetomidine affects cerebral activity in preterm infants Cortes-Ledesma, Cristina; Arruza, Luis; Sainz-Villamayor, Angela ...
Archives of disease in childhood. Fetal and neonatal edition,
05/2023, Letnik:
108, Številka:
3
Journal Article
Recenzirano
The use of dexmedetomidine (DEX) has been extended in preterm newborns, but the effects on cerebral activity and their relationship with haemodynamic changes has not been studied.We retrospectively ...studied the effects of DEX administered to 10 preterm newborns, assessing amplitude-integrated EEG (aEEG) parameters, brain regional SO
(brSO
), heart rate, non-invasive mean blood pressure (MBP), transcutaneous oxygen saturation (SpO
), venous pCO2 and haemoglobin (Hb) values, in two 6-hour periods: one starting 6 hours before the beginning of DEX perfusion and the other 6 hours afterwards.DEX infusion led to brSO
decrease not associated to heart rate, MBP, SpO
, Hb or pCO2 variation, which suggests that brSO
decrease could be related to local vasoconstriction. DEX infusion led to prolongation of interburst interval and reduction of cycling. Such effects, not been described so far, should be considered in the assessment of aEEG traces after DEX administration to avoid misinterpretations regarding patient's prognosis. More studies are needed to assess the safety of DEX use in the newborn.
Currently, there is a lack of effective treatments or preventive strategies for bronchopulmonary dysplasia (BPD). Pre-clinical studies with mesenchymal stromal cells (MSCs) have yielded encouraging ...results. The safety of administering repeated intravenous doses of umbilical cord tissue-derived mesenchymal stromal cells (UC-MSCs) has not yet been tested in extremely-low-gestational-age newborns (ELGANs).
to test the safety and feasibility of administering three sequential intravenous doses of UC-MSCs every 7 days to ELGANs at risk of developing BPD. Methods: In this phase 1 clinical trial, we recruited ELGANs (birth weight ≤1250 g and ≤28 weeks in gestational age GA) who were on invasive mechanical ventilation (IMV) with FiO2 ≥ 0.3 at postnatal days 7–14. Three doses of 5 × 106/kg of UC-MSCs were intravenously administered at weekly intervals. Adverse effects and prematurity-related morbidities were recorded.
From April 2019 to July 2020, 10 patients were recruited with a mean GA of 25.2 ± 0.8 weeks and a mean birth weight of 659.8 ± 153.8 g. All patients received three intravenous UC-MSC doses. The first dose was administered at a mean of 16.6 ± 2.9 postnatal days. All patients were diagnosed with BPD. All patients were discharged from the hospital. No deaths or any serious adverse events related to the infusion of UC-MSCs were observed during administration, hospital stays or at 2-year follow-up.
The administration of repeated intravenous infusion of UC-MSCs in ELGANs at a high risk of developing BPD was feasible and safe in the short- and mid-term follow-up.
Abstract
Objective
To compare the influence of aPLs on global and cardiovascular damage in patients with SLE diagnosed before and after the year 2000.
Methods
Two hundred and eighty-six patients from ...the Lupus-Cruces cohort with a minimum follow-up of 5 years were divided into two subcohorts according to the date of diagnosis, before 2000 (less than 2000) and from 2000 on (2000 or more). We compared the mean Systemic Lupus Erythematosus International Collaborating Clinics-American College of Rheumatology (SLICC-ACR) Damage Index score and global and cardiovascular damage-free survival rates in the presence or absence of aPL in both subcohorts. Variables potentially modulating damage among aPL-positive patients were analysed.
Results
The subcohorts were comparable for demographic and lupus-related variables except for treatment variables: the 2000 or more subcohort received lower doses of prednisone and more HCQ, low-dose aspirin, statins, immunosuppressive agents and vitamin D. aPL-positive patients in the less than 2000, but not in the 2000 or more subcohort, accrued more damage compared with aPL-negative patients. In the less than 2000 subcohort, the adjusted hazard ratios (HRs) for global and cardiovascular damage in aPL-positive vs aPL-negative patients were 1.98 (95% CI 1.24, 3.14) and 9.3 (95% CI 3.24, 26.92), respectively. No differences in damage were seen between aPL-positive and aPL-negative patients in the 2000 or more subcohort. Hypertension (HR = 4.64, 95% CI 1.33, 16.19), LA (HR = 3.85, 95% CI 1.1, 13.41) and the number of months on HCQ (HR = 0.97, 95% CI 0.95, 0.99) were independent predictors of vascular damage in the combined analysis of all aPL-positive patients.
Conclusion
The effects of aPL on damage accrual in SLE patients have been reduced over recent years. The widespread use of HCQ and a better thromboprophylaxis are likely causing this change.
Despite advances in respiratory distress syndrome (RDS) management over the past decade, non-invasive ventilation (NIV) failure is frequent and associated with adverse outcomes. There are ...insufficient data on the failure of different NIV strategies currently used in clinical practice in preterm infants.
This was a prospective, multicenter, observational study of very preterm infants gestational age (GA) <32 weeks admitted to the neonatal intensive care unit for RDS that required NIV from the first 30 min after birth. The primary outcome was the incidence of NIV failure, defined as the need for mechanical ventilation for <72 h of life. Secondary outcomes were risk factors associated with NIV failure and complication rates.
The study included 173 preterm infants with a median GA of 28 (IQR 27-30) weeks and a median birth weight of 1,100 (IQR 800-1,333) g. The incidence of NIV failure was 15.6%. In the multivariate analysis, lower GA (OR, 0.728; 95% CI, 0.576-0.920) independently increased the risk of NIV failure. Compared to NIV success, NIV failure was associated with higher rates of unfavorable outcomes, including pneumothorax, intraventricular hemorrhage, periventricular leukomalacia, pulmonary hemorrhage, and a combined outcome of moderate-to-severe bronchopulmonary dysplasia or death.
NIV failure occurred in 15.6% of the preterm neonates and was associated with adverse outcomes. The use of LISA and newer NIV modalities most likely accounts for the reduced failure rate. Gestational age remains the best predictor of NIV failure and is more reliable than the fraction of inspired oxygen during the first hour of life.
Postnatal steroids, often used to prevent and treat bronchopulmonary dysplasia, may influence the growth of preterm infants, although data are scarce in the literature. This is a multicenter cohort ...study including surviving preterm infants <32 weeks at birth (
= 17,621) from the Spanish Neonatal Network SEN1500 database, without major congenital malformations. Linear regression models were adjusted for postnatal steroids, respiratory severity course (invasive mechanical ventilation at 28 days), progression to moderate-severe bronchopulmonary dysplasia (O
at 36 weeks), length of stay, sex, gestational age and z-scores at birth. A subgroup analysis depending on the timing of administration, ventilation status at 28 days and moderate-severe BPD diagnosis was also performed. Overall, systemic postnatal steroids were not independently associated with poorer weight gain (0.1; 95% CI: -0.05 to 0.2 g/kg/day), linear growth (0; 95% CI: -0.03 to 0.01 cm/week) or head circumference growth (-0.01; 95% CI: -0.02 to 0 cm/week). Patients who received steroids after 28 days or who were not O
dependent at 36 weeks after having received steroids gained more weight (0.22; 95% CI: 0.04 to 0.4 and 0.2; 95% CI: 0.004 to 0.5 g/kg/day, respectively). Globally, systemic postnatal steroids had no significant adjusted effect on postnatal growth.
Aim
We describe the postnatal weight gain, linear and head growth trends of surviving preterm infants from 2005 to 2017.
Methods
Multicentre cohort study, including surviving preterm infants ...<32 weeks (n = 21 084), from the Spanish Neonatal Network database, without major congenital malformations who were less than 50 weeks postmenstrual age at discharge. Outcomes were weight gain (g/kg/day), linear and head growth (cm/week) and changes in weight, length and head circumference z‐scores from birth to discharge. The study period was divided into 2005–8, 2009–11, 2012–14 and 2015–17.
Results
Weight gain, linear growth and head growth were slightly higher in 2015–2017 than in 2005–2008: 12.2 ± 2.6 to 13.1 ± 2.5 g/kg/day, 0.98 ± 0.6 to 1.03 ± 0.6 cm/week and 0.76 ± 0.2 to 0.77 ± 0.3 cm/week, respectively. It was associated with a decreased fall in weigh, length and head circumference z‐scores from birth to discharge (−1.32 ± 0.9 to −1.01 ± 0.84, −1.38 ± 1.2 to −1.18 ± 1.2 and −0.41 ± 1.2 to −0.33 ± 1.3, respectively).
Conclusion
Postnatal growth restriction remained a common complication of prematurity despite some increment over the last years. Growth disproportionality seemed to be worsening as weight gain was increased more than linear growth.
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