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Pediatric Thyroidectomy: Experience From a Portuguese Hospital
Full text linkBackground and objective Pediatric thyroid disease requiring surgery is rare. Thyroid nodules are a frequent indication for surgery and are mostly benign. However, up to 25% of cases can be malignant. In this study, we aimed to describe our center's experience with regard to pediatric thyroid surgery. Methods This was a retrospective transverse study involving pediatric patients who underwent thyroid surgery at a tertiary hospital between January 2010 and December 2021. Results A total of 14 patients underwent 15 surgeries. The main reason for referral to pediatric endocrinology was thyroid nodules (n=10). Thirteen fine needle aspirations (FNAs) were performed, with follicular tumor (n=6) being the most common finding. The median age of patients at surgery was 15.9 years [interquartile range (IQR): 14.0-16.8]. The most common surgical indications were the presence of a follicular tumor on FNA (n=5) and thyroid nodule size causing symptoms (n=5). There was one case of prophylactic thyroidectomy due to the identification of a multiple endocrine neoplasia type 2A (MEN2A) mutation. The most frequently described histopathology results were follicular adenoma (n=6) and colloid nodular goiter (n=6). Three postoperative complications were observed in three different patients: bilateral lesion of the recurrent laryngeal nerve, cervical hematoma, and transient hypoparathyroidism with hypocalcemia. Conclusion In our study, the most frequent surgical indication was a follicular tumor. A good correlation was found between FNA cytology and final histopathology results, which is in accordance with previous studies. This reinforces the importance of FNA in diagnosis and surgical planning. The rate of complications in our study is comparable to that in larger single-center series in the literature.info:eu-repo/semantics/publishedVersio
Temporary atrial septal defect balloon occlusion test as a must in the elderly
Full text linkBackground: Atrial septal defect (ASD) can often remain asymptomatic until adulthood. It still remains unclear whether large ASD closure in senior people should be performed or not. Temporary ASD balloon occlusion test has been suggested as a tool to assess the risk of acute left ventricular heart failure post-ASD closure, and it allows to better distinguish responders from non-responders.
Case presentation: An 83-year-old man with a long-standing uncorrected secundum ASD was admitted for recently decompensated right-sided heart failure. During hospitalization, this patient was studied with trans-esophageal echocardiography, cardiac magnetic resonance imaging, and right heart catheterization, showing high Qp:Qs ratio and favorable anatomical conditions for percutaneous closure. Because of patient's increasing need for intravenous diuretics and worsening renal function, it was considered that transcatheter ASD closure could improve symptoms, hence it was performed an attempt of percutaneous closure of the ASD with a fenestrated device. Unfortunately, irrespective of ASD being hemodynamically significant, it was found a very significant increase in pulmonary capillary wedge pressure during the temporary balloon occlusion test, supporting the existence of concealed left ventricular diastolic dysfunction. As a result, it was decided to abandon the procedure and not to close the ASD.
Conclusion: This clinical case illustrates the value of temporary balloon occlusion test before permanent percutaneous closure of ASD in elderly patients, regardless of left ventricular (systolic or diastolic) dysfunction.info:eu-repo/semantics/publishedVersio
Living Donors’ Age Modifies the Impact of Pre-Donation Estimated Glomerular Filtration Rate on Graft Survival
Full text linkBackground: The global scarcity of organs for kidney transplants (KTs) has led to the increased acceptance of living donors (LDs) with minor abnormalities to increase the donor pool.. We sought to evaluate the effects of some of these LDs' clinical characteristics (older age, borderline renal function, hypertension, dyslipidemia, smoking, and obesity) on graft outcomes.
Methods: We studied 352 recipients of LDKTs (1998-2020). Firstly, considering the recipients and KT variables, we identified relevant predictors of overall and censored graft failure (GF). Then, adjusting for these predictors, we explored LD variables as predictors of overall and censored GF in a multivariable Cox model.
Results: The recipients from LD with higher eGFR (≥90 mL/min/1.73 m2) had significantly better overall and censored graft survival GS) at 15 y after KT (respectively, 67 and 75% vs. 46 and 46%, p < 0.001). Importantly, none of the remaining LD factors which were evaluated (hypertension, dyslipidemia, smoking, proteinuria, and obesity) were independent predictors of GF. In recipients from LDs < 50 y, having an eGFR < 90 was an independent predictor of overall GF [adjusted HR (95%CI) of 2.578 (1.120-5.795)] and censored GF [adjusted HR (95%CI) of 3.216 (1.300-7.959)], compared to recipients from LDs with eGFR ≥ 90. Contrarily, when donors were older, no difference in the risk of GF was observed between eGFR categories.
Conclusion: In our cohort, lower pre-donation eGFR had an impact on GS only in younger LDs. An age-adjusted eGFR cutoff may be pursued for improved donor admissibility.info:eu-repo/semantics/publishedVersio
Use of albumin infusion for cirrhosis-related complications: An international position statement
Full text linkBackground & aims: Numerous studies have evaluated the role of human albumin (HA) in managing various liver cirrhosis-related complications. However, their conclusions remain partially controversial, probably because HA was evaluated in different settings, including indications, patient characteristics, and dosage and duration of therapy.
Methods: Thirty-three investigators from 19 countries with expertise in the management of liver cirrhosis-related complications were invited to organise an International Special Interest Group. A three-round Delphi consensus process was conducted to complete the international position statement on the use of HA for treatment of liver cirrhosis-related complications.
Results: Twelve clinically significant position statements were proposed. Short-term infusion of HA should be recommended for the management of hepatorenal syndrome, large volume paracentesis, and spontaneous bacterial peritonitis in liver cirrhosis. Its effects on the prevention or treatment of other liver cirrhosis-related complications should be further elucidated. Long-term HA administration can be considered in specific settings. Pulmonary oedema should be closely monitored as a potential adverse effect in cirrhotic patients receiving HA infusion.
Conclusions: Based on the currently available evidence, the international position statement suggests the potential benefits of HA for the management of multiple liver cirrhosis-related complications and summarises its safety profile. However, its optimal timing and infusion strategy remain to be further elucidated.Impact and implications: Thirty-three investigators from 19 countries proposed 12 position statements on the use of human albumin (HA) infusion in liver cirrhosis-related complications. Based on current evidence, short-term HA infusion should be recommended for the management of HRS, LVP, and SBP; whereas, long-term HA administration can be considered in the setting where budget and logistical issues can be resolved. However, pulmonary oedema should be closely monitored in cirrhotic patients who receive HA infusion.info:eu-repo/semantics/publishedVersio
Vaccine breakthrough hypoxemic COVID-19 pneumonia in patients with auto-Abs neutralizing type I IFNs
Full text linkLife-threatening "breakthrough" cases of critical COVID-19 are attributed to poor or waning antibody (Ab) response to SARS-CoV-2 vaccines in individuals already at risk. Preexisting auto-Abs neutralizing type I IFNs underlie at least 15% of critical COVID-19 pneumonia cases in unvaccinated individuals; their contribution to hypoxemic breakthrough cases in vaccinated people is unknown. We studied a cohort of 48 individuals (aged 20 to 86 years) who received two doses of a messenger RNA (mRNA) vaccine and developed a breakthrough infection with hypoxemic COVID-19 pneumonia 2 weeks to 4 months later. Ab levels to the vaccine, neutralization of the virus, and auto-Abs to type I IFNs were measured in the plasma. Forty-two individuals had no known deficiency of B cell immunity and a normal Ab response to the vaccine. Among them, 10 (24%) had auto-Abs neutralizing type I IFNs (aged 43 to 86 years). Eight of these 10 patients had auto-Abs neutralizing both IFN-α2 and IFN-ω, whereas two neutralized IFN-ω only. No patient neutralized IFN-β. Seven neutralized type I IFNs at 10 ng/ml and three at 100 pg/ml only. Seven patients neutralized SARS-CoV-2 D614G and Delta efficiently, whereas one patient neutralized Delta slightly less efficiently. Two of the three patients neutralizing only type I IFNs at 100 pg/ml neutralized both D614G and Delta less efficiently. Despite two mRNA vaccine inoculations and the presence of circulating Abs capable of neutralizing SARS-CoV-2, auto-Abs neutralizing type I IFNs may underlie a notable proportion of hypoxemic COVID-19 pneumonia cases, highlighting the importance of this particularly vulnerable population.info:eu-repo/semantics/publishedVersio
Real-World Experience in Treatment of Patients with Non-Small-Cell Lung Cancer with BRAF or cMET Exon 14 Skipping Mutations
Full text linkBRAF and cMET exon 14 skipping are rare mutations of NSCLC. The treatment sequence in these cases for the first and second line is not clear. An international registry was created for patients with advanced NSCLC harboring BRAF or cMET exon 14 skipping mutations, diagnosed from January 2017 to June 2022. Clinicopathological and molecular data and treatment patterns were recorded. Data on 58 patients, from eight centers across five countries, were included in the final analysis. We found that 40 patients had the cMET exon 14 skipping mutation and 18 had the BRAF V600E mutation. In total, 53 and 28 patients received first- and second-line treatments, respectively, among which 52.8% received targeted therapy (TT) in the first line and 53.5% in the second line. The overall response rate (ORR) and disease control rate (DCR) for first-line treatment with TT vs. other treatment such as immune checkpoint inhibitors ± chemotherapy (IO ± CT) were 55.6% vs. 21.7% (p = 0.0084) and 66.7% vs. 39.1% (p = 0.04), respectively. The type of treatment in first-line TT vs. other affected time to treatment discontinuation (TTD) was 11.6 m vs. 4.6 m (p= 0.006). The overall survival for the whole group was 15.4 m and was not statistically affected by the type of treatment (19.2 m vs. 13.5 m; p = 0.83).info:eu-repo/semantics/publishedVersio
Effect of Anesthesia on Endoscopic Sinus Surgery Hemostasis: a State-of-the-Art Review
Full text linkEndoscopic sinus surgery (ESS) is the gold standard for the management of chronic rhinosinusitis, nasal polyposis, and other pathology involving paranasal sinus as tumors. Intraoperative bleeding during ESS can be challenging due to the narrow sinonasal surgical field, single working hand, and the use of endoscopic instruments, which may affect hemostasis. There is a role for the type of anesthesia technique used for intraoperative bleeding control. Total intravenous anesthesia (TIVA) and inhalational anesthesia (IA) are some of the techniques available for anesthetic purposes. While both techniques have their advantages and disadvantages, there is a need to compare their efficacy and safety to determine which technique is more appropriate for ESS. In this review, our main focus was to summarize the current evidence about the different techniques of anesthesia used during ESS. A systematic review of the PubMed/MEDLINE database was performed using specific English terms related to TIVA and IA/volatile anesthesia used during ESS. A total of 548 publications were considered. Among these, 329 studies did not fulfill the criteria for inclusion in the systematic review, resulting in the inclusion of only 132 publications: 13 systematic reviews, 32 reviews, 92 randomized controlled trials, and 13 meta-analyses. The state of the art favors the use of TIVA during ESS due to its significant improvement in the intraoperative surgical field with less blood loss. Further studies aim to compare long-term nasal status with objective tools, ideally in similar pathology with the same surgeon.info:eu-repo/semantics/publishedVersio
Domiciliary High-Flow Nasal Therapy in Primary Ciliary Dyskinesia
Full text linkWe report the case of an adolescent with severe primary ciliary dyskinesia (PCD) phenotype associated with a rare genotype. His clinical condition deteriorated, with daily cough and breathlessness, hypoxemia, and lung function decline. Despite being started on home noninvasive ventilation (NIV), the symptoms progressed to dyspnea at rest and thoracic pain. High-flow nasal cannula (HFNC) was started during the daytime as an adjuvant to NIV, and he was started on regular oral opioids for pain and dyspnea control. There was a clear improvement in comfort and dyspnea and breathing work relief. Additionally, better exercise tolerance was also noted. He is currently on the lung transplant waiting list. We aim to emphasize the benefits of HFNC as an add-on therapy for the management of chronic breathlessness since our patient experienced an improvement in breathing and exercise tolerance. However, there is a paucity of studies regarding domiciliary HFNC, particularly in pediatric age. Therefore, further studies are needed to achieve optimal and personalized care. Close monitoring and frequent reassessment in a specialized center are key to adequate management.info:eu-repo/semantics/publishedVersio
Ambiguous Genitalia: An Unexpected Diagnosis in a Newborn
Full text linkAlterations in gonad formation or function can lead to congenital conditions in which chromosomal, gonadal, or anatomical sex is atypical. These conditions are referred to as disorders of sex development (DSD) and have a heterogeneous etiology. The assessment of these children by a multidisciplinary team is crucial for an accurate diagnosis and should be initiated promptly due to the potentially life-threatening nature of congenital adrenal hyperplasia, a common cause of DSD. We present a neonate born at 39 weeks with a weak cry, slight hypotonia, poor suction reflex, peculiar facies, and ambiguous genitalia. From the study carried out, the abdominopelvic ultrasound revealed a nodular structure compatible with the left gonad. Aneuploidy screening confirmed the presence of the Y chromosome. Additionally, normal endocrinological studies and the karyotype showed a genotype compatible with cri-du-chat syndrome with partial trisomy of chromosome 3. Children with cri-du-chat syndrome characteristically exhibit a cat-like cry and distinctive facial features, along with developmental delay and intellectual disability. Duplication of 3p is a rare genetic disorder, usually associated with other chromosomal anomalies and congenital malformations, namely, of the genitalsinfo:eu-repo/semantics/publishedVersio
Acute Fulminant Cerebral Edema in a Child With Suspected Meningoencephalitis
Full text linkAcute fulminant cerebral edema (AFCE) is a recently identified encephalitis type associated with significant morbimortality. Described as rare, limited data exists on its early detection and treatment. This paper describes a case of AFCE that progressed to unresponsive intracranial hypertension. A previously healthy four-year-old boy presented with fever, myalgias, and neurological symptoms. Diagnostic assessments showed cerebrospinal fluid abnormalities, and despite medical interventions, his condition deteriorated rapidly and developed severe cerebral edema and herniation within 24 hours. A decompressive craniectomy was attempted to decrease intracranial pressure, without success. This case emphasizes the urgency of early AFCE recognition and effective management strategies given its severe prognosis, aiming to improve understanding and spur further researchinfo:eu-repo/semantics/publishedVersio