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Mucosal Immunology Apr 2024Sublingual allergen immunotherapy (SLIT) is an emerging treatment option for allergic asthma and a potential disease-modifying strategy for asthma prevention. The key...
Sublingual allergen immunotherapy (SLIT) is an emerging treatment option for allergic asthma and a potential disease-modifying strategy for asthma prevention. The key cellular events leading to such long-term tolerance remain to be fully elucidated. We administered prophylactic SLIT in a mouse model of house dust mite (HDM)-driven allergic asthma. HDM extract was sublingually administered over 3 weeks followed by intratracheal sensitization and intranasal challenges with HDM. Prophylactic SLIT prevented allergic airway inflammation and hyperreactivity with a low lab-to-lab variation. The HDM-specific T helper (Th)2 (cluster of differentiation 4 Th) response was shifted by SLIT toward a regulatory and Th17 response in the lung and mediastinal lymph node. By using Derp1-specific cluster of differentiation 4 T cells (1-DER), we found that SLIT blocked 1-DER T cell recruitment to the mediastinal lymph node and dampened IL-4 secretion following intratracheal HDM sensitization. Sublingually administered Derp1 protein activated 1-DER T cells in the cervical lymph node via chemokine receptor7 migratory dendritic cells (DC). DCs migrating from the oral submucosa to the cervical lymph node after SLIT-induced Foxp3 regulatory T cells. When mice were sensitized with HDM, prior prophylactic SLIT increased Derp1 specific regulatory T cells (Tregs) and lowered Th2 recruitment in the lung. By using Foxp3-diphtheria toxin receptor mice, Tregs were found to contribute to the immunoregulatory prophylactic effect of SLIT on type 2 immunity. These findings in a mouse model suggest that DC-mediated functional Treg induction in oral mucosa draining lymph nodes is one of the driving mechanisms behind the disease-modifying effect of prophylactic SLIT.
PubMed: 38570140
DOI: 10.1016/j.mucimm.2024.03.012 -
International Journal of Surgery Case... Apr 2024Primary pleural hydatid cysts (PPHCs) are a rare clinical condition caused by the larval stage of the parasite Echinococcus granulosus. They occur in <1 % of all...
INTRODUCTION
Primary pleural hydatid cysts (PPHCs) are a rare clinical condition caused by the larval stage of the parasite Echinococcus granulosus. They occur in <1 % of all hydatid cysts in the body and can cause serious complications such as pneumothorax, pleural effusion, and mediastinal shift.
PRESENTATION OF CASE
We report a rare case of a 28-year-old female who was initially misdiagnosed and ignored her pneumothorax for several months, resulting in progressive dyspnea and chest pain. After performing radiological images, a primary pleural hydatid cyst was suspected. She was surgically treated and the cyst was removed by our doctors and the patient improved without any significant complications.
DISCUSSION
PPHCs are a challenging diagnosis due to their nonspecific symptoms and low prevalence. They can mimic other pleural diseases such as tuberculosis, empyema, or malignancy. The diagnosis of PPHCs requires a high index of suspicion and a combination of imaging, serology, and histopathology. The treatment of choice is surgical removal of the cyst, along with perioperative anthelmintic therapy to prevent recurrence and anaphylaxis.
CONCLUSION
PPHCs are a rare but potentially life-threatening condition that requires early diagnosis and management. Clinicians should be aware of this entity and include it in the differential diagnosis of pleural diseases, especially in developing countries. Surgical treatment is effective and safe, and can improve the quality of life of patients with PPHCs.
PubMed: 38452642
DOI: 10.1016/j.ijscr.2024.109463 -
Andes Pediatrica : Revista Chilena de... Dec 2023Pleuropulmonary blastoma (PPB) is the most common pediatric malignant primary lung tumor. It's associated with the DICER1 gene pathogenic germline variants. Antenatal...
UNLABELLED
Pleuropulmonary blastoma (PPB) is the most common pediatric malignant primary lung tumor. It's associated with the DICER1 gene pathogenic germline variants. Antenatal presentation is infrequent and poses a challenge in the differential diagnosis of congenital pulmonary airway malformation (CPAM).
OBJECTIVE
to report a case of unusual presentation of PPB associated with DICER1 syndrome and to describe the difficulty in differentiating it from CPAM.
CLINICAL CASE
Male patient with prenatal diagnosis of hypervascular left lung lesion, with mediastinal shift and progressive growth, initially interpreted as CPAM. He was born at 38 weeks, requiring transitory treatment with positive pressure due to ventilatory impairment. A CT scan with contrast showed a large multilocular cystic mass containing air causing mass effect, requiring open left upper lobectomy. Histology results were compatible with type I PPB, with negative margins, and positive genetic study for DICER1 syndrome. Seven weeks post-resection, an aerial image was detected in the upper left side of the chest, with progressive growth, requiring a new tumor resection and upper segmentectomy, with biopsy corresponding to recurrence of type I PPB with negative margins. He received adjuvant treatment with chemotherapy, with follow-up for 2 years, remaining asymptomatic, without recurrence, and with negative screening for other neoplasms associated with DICER1 syndrome. Among the family history, the mother had papillary thyroid cancer and tested positive for the mutation.
CONCLUSION
PPB is a rare cancer, difficult to distinguish from CPAM, especially in its antenatal presentation. Nowing its association with DICER1 syndrome and performing a genetic study are key to the early detection of BPP and the search for other tumors associated with the syndrome.
Topics: Female; Humans; Male; Pregnancy; DEAD-box RNA Helicases; Diagnosis, Differential; Lung; Lung Neoplasms; Pulmonary Blastoma; Ribonuclease III; Infant, Newborn
PubMed: 38329309
DOI: 10.32641/andespediatr.v94i6.4663 -
Mediastinum (Hong Kong, China) 2024Locally invasive thymic neoplasms are challenging clinical scenarios and typically require a multidisciplinary approach. The involvement of major mediastinal veins such... (Review)
Review
Locally invasive thymic neoplasms are challenging clinical scenarios and typically require a multidisciplinary approach. The involvement of major mediastinal veins such as the superior vena cava (SVC) used to be a contraindication to surgery, but with improved surgical technique and outcomes, this paradigm has shifted. In some situations, complex resections and reconstructions may be indicated and required to improve the long-term outcome of these patients. We report two of our cases along with a current review of literature. We also describe the preoperative workup, operative techniques, postoperative management, complications, and outcomes of patients with invasive thymic neoplasms that involve the mediastinal veins. Our first case describes a patient who was diagnosed with a thymoma extending from the diaphragm to the base of the neck that was also encasing major vascular structures including the SVC and left innominate vein. Our second case describes a patient who was also diagnosed with a large anterior mediastinal mass encasing the great veins and invading the chest wall. We describe the management of these patients and then delve deeper into operative techniques including SVC resection and reconstruction. We describe the types of conduits that can be used and complications to be mindful of when clamping the great veins, such as the SVC. Improvements in conduit materials and neoadjuvant and adjuvant therapies over the years have made it more feasible for patients with invasive thymic neoplasms to undergo surgery.
PubMed: 38322190
DOI: 10.21037/med-20-69 -
A Canadian children's hospital's experience with cystic echinococcosis over 30 years: A case series.Journal of the Association of Medical... Jan 2024Cystic echinococcosis (CE) or hydatid disease caused by the cestode is an uncommon infection in Canada especially among children. There are limited reports describing...
BACKGROUND
Cystic echinococcosis (CE) or hydatid disease caused by the cestode is an uncommon infection in Canada especially among children. There are limited reports describing the clinical presentation and management in Canadian children.
METHODS
The medical records of all children diagnosed with CE at a quaternary paediatric centre in Ontario between January 1988 and August 2021 were retrospectively reviewed. The clinical course, management, and outcomes of each case were summarized.
RESULTS
We report two paediatric cases of cystic echinococcosis (CE) in detail and review four additional cases seen at our institution over 33.5 years. The first case was a previously healthy 12-year-old boy with pulmonary CE resulting in unilateral lung collapse and mediastinal shift, who was presumedly infected while living in the Middle East. The second case was a previously healthy 3-year-old girl with pulmonary CE acquired locally in southern Ontario. Four other cases of CE with hepatic involvement (median age 12.5 years) were identified during the study period. Five out of six patients received both surgical and medical therapy.
CONCLUSION
CE is a rare but serious disease seen in southern Canada that has historically been associated with travel or migration. Due to changes in urban wildlife landscapes and increased global migration, CE may become more prevalent in Canadian children. We describe the first locally acquired case in rural southern Ontario diagnosed at our centre. Prompt recognition of this infection in children by health care providers is important to prevent morbidity and mortality.
PubMed: 38250618
DOI: 10.3138/jammi-2022-0041 -
Journal of Clinical Medicine Jan 2024Fetal magnetic resonance imaging (MRI) is broadly used as a method for assessing prognosis in congenital diaphragmatic hernia (CDH). In addition to the extent of lung...
OBJECTIVE
Fetal magnetic resonance imaging (MRI) is broadly used as a method for assessing prognosis in congenital diaphragmatic hernia (CDH). In addition to the extent of lung hypoplasia, determined by measuring the lung volume, cardiac impairment due to pulmonary hypertension and left cardiac hypoplasia is decisive for the prognosis. The percentage area of left ventricle (pALV) describes the percentage of the inner area of the left ventricle in relation to the total area, whereas the mediastinal shift angle (MSA) quantifies the extent of cardiac displacement. The prognostic value of pALV and MSA should be evaluated in terms of survival, the need for extracorporeal membrane oxygenation (ECMO) therapy, and the development of chronic lung disease (CLD).
METHODS
In a total of 122 fetal MRIs, the MSA and pALV were measured retrospectively and complete outcome parameters were determined regarding survival for all 122 subjects, regarding ECMO therapy in 109 cases and about the development of CLD in 78 cases. The prognostic value regarding the endpoints was evaluated using logistic regression and ROC analysis.
RESULTS
The MSA was significantly higher in children who received ECMO therapy ( = 0.0054), as well as in children who developed CLD ( = 0.0018). ROC analysis showed an AUC of 0.68 for ECMO requirement and 0.77 with respect to CLD development. The pALV showed a tendency towards higher levels in children who received ECMO therapy ( = 0.0824). The MSA and the pALV had no significant effect on survival (MSA: = 0.4293, AUC = 0.56; pALV: = 0.1134, AUC = 0.57).
CONCLUSIONS
The MSA determined in fetal MRI is a suitable prognostic parameter for ECMO requirement and CLD development in CDH patients and can possibly be used as a supplement to the established parameters.
PubMed: 38202274
DOI: 10.3390/jcm13010268 -
Cureus Dec 2023Morgagni hernia is a rare congenital defect of the diaphragm, especially seen in children but rarely observed in adults. It occurs due to a congenital defect during the...
Morgagni hernia is a rare congenital defect of the diaphragm, especially seen in children but rarely observed in adults. It occurs due to a congenital defect during the development of the diaphragm. Bochdalek hernia is a common congenital form of diaphragmatic hernia. Morgagni hernia is usually rare with a prevalence of approximately 2-3%. Beaver tail liver, which is also called sliver of liver, is a rare variant of liver morphology. Sometimes elongated left lobe of the liver can extend laterally across the midline to contact and often surround the spleen. A 46-year-old female from Karnataka complained of fever with chills and lower back aches for seven days with no history of chest pain, vomiting, or diarrhea. There have been no similar complaints in the past. She had no other comorbidities. She was a non-smoker and non-alcoholic. Biomass gas exposure for 20 years was noted as she cooked food with firewood. She was a housewife by occupation with no history of trauma or surgeries in the past. The general physical exam was unremarkable. The respiratory system was normal. Auscultation showed decreased breath sounds in the mammary area of the right side of the chest with normal vesicular breath sounds in all other areas. Per abdominal exam showed a flat abdomen. Umbilicus was central in position. There was tenderness in the right hypochondriac and epigastric regions with no guarding or rigidity. On examining the cardiovascular system, apical impulses could not be palpated and normal heart sounds were heard with no cardiac murmurs. Other systems examination was normal. Routine blood investigations were done, revealing hemoglobin of 11.6%, total WBC of 6270 cells, and hematocrit of 33.1%. The renal function test was within normal limits (creatinine = 0.7 mg/dl). A chest X-ray revealed a right lower zone, para cardiac well-circumscribed structure suggestive of a cyst with an air-fluid level inside. Chest CT was suggestive of a hernia in the anterior aspect of the diaphragm measuring 3.5 x 3.3 cm at the level of D9 vertebral body with transverse colon and omentum as its contents, ascending upwards into anterior and superior mediastinum for a length of 13 cm causing shift of cardia posteriorly and to the left (anterior midline diaphragmatic hernia - Morgagni hernia). A hyperdense lesion (Hounsfield unit = 64) measuring 1.3 x 1.8 cm was noted in segment seven of the right lobe of the liver, suggestive of a complex cyst. Beaver tail was noted in the liver. Morgagni hernia usually presents in younger age groups with respiratory symptoms. Its incidental detection in adults is very rare. In this case, the patient was having lower backache and no other gastrointestinal symptoms. The respiratory and cardiothoracic systems get affected because the intestinal contents herniating through the diaphragm shift the position of the cardia and the lower lobes of the lungs, which may have implications such as repeated cough and infections. Symptomatic hernias are usually detected in an early age group. It can present with symptoms of gastrointestinal obstruction or acute chest symptoms or can even be asymptomatic. Treatment is primarily surgical repair of the hernia. This can be done either transthoracically or transabdominally. It is usually advised that surgical repair should be done even in asymptomatic cases as in this case, to avoid obstruction of the intestine or worsening of the hernia that is pulling the abdominal contents into the thorax.
PubMed: 38161555
DOI: 10.7759/cureus.49769 -
JTCVS Techniques Dec 2023Branch-first total aortic arch repair is a paradigm shift in the technical approach for uninterrupted neuroprotection during open aortic surgery. This technique is...
BACKGROUND
Branch-first total aortic arch repair is a paradigm shift in the technical approach for uninterrupted neuroprotection during open aortic surgery. This technique is further modified to instigate hazardous sternal reentry in patients with hostile mediastinal anatomy at risk of aortic injury.
METHODS
Intraoperative preparation and the illustrated operative technique of the cervical branch-first technique are described. The accompanying case series narrates the experiences and outcomes of 4 patients who underwent successful complex reoperative aortic surgery utilizing this technique.
RESULTS
The indications for resternotomy included a sixth reoperation for recurrent mycotic aortic pseudoaneurysm, a third reoperation for extensive infective endocarditis, a reoperation for complete Bentall graft dehiscence with contained aortic rupture, and a third reoperation for residual type A dissection. All patients survived their proposed surgery. Two patients were operated on in an emergency setting. Two patients separated from cardiopulmonary bypass with extracorporeal support. None experienced permanent neurological sequelae, gut ischemia, peripheral arterial complications, or in-hospital mortality. One mortality due to decompensated heart failure was reported at 6 months postoperatively.
CONCLUSIONS
The cervical branch-first technique offers unparalleled advantage in neuroprotection from an early stage of complex reoperative aortic surgery. It provides an independent circuit for complete antegrade cerebral perfusion, irrespective of suspension to circulatory flows to the rest of the body during complex reentry into hostile chests. Our experience to date has demonstrated promising outcomes and further refinements will guide patient selection best suited for this technique.
PubMed: 38152225
DOI: 10.1016/j.xjtc.2023.08.030 -
Life (Basel, Switzerland) Dec 2023Unilateral proximal interruption of the pulmonary artery (UPIPA) is a rare congenital disease, and its optimal management remains undefined in the existing literature.... (Review)
Review
BACKGROUND
Unilateral proximal interruption of the pulmonary artery (UPIPA) is a rare congenital disease, and its optimal management remains undefined in the existing literature. The occasional necessity for pneumonectomy is still supported by limited evidence.
METHODS
A systematic review of the literature was conducted using the PubMed search engine, focusing on UPIPA cases that received pneumonectomy. Thirty-one pertinent articles were selected and included in the analysis. A case reported from our institution was included in the analysis.
RESULTS
We found 25 adults and seven children affected by UPIPA who received an indication for pneumonectomy, plus an additional case that was reported by our institution. Among adult patients, the predominant indication was hemoptysis (57%), followed by suspected or confirmed lung cancer (23%). Approximately 46% of surgical procedures were classified as urgent or emergent. Postoperative complications were observed in 36% of cases, with no recorded mortality. In pediatric cases, pneumonectomy was primarily a life-saving intervention, performed urgently or emergently in 75% of instances. A possible late complication in pediatric patients involves a mediastinal shift leading to respiratory distress, which may be mitigated using an inflatable prosthesis.
CONCLUSIONS
Pneumonectomy achieves complete resolution of UPIPA symptoms. In the adult population, its primary indication is hemoptysis, with procedures conducted in both elective and urgent/emergent settings. Despite a mortality rate of zero, a notable proportion of patients may experience postoperative complications. In pediatric cases, the clinical presentation varies more extensively, and pneumonectomy is typically reserved for life-threatening situations, emphasizing the need for careful patient selection.
PubMed: 38137929
DOI: 10.3390/life13122328