Rare broncho-pulmonary arterial fistula in a healthy 9-year-old girl

2020 ◽  
Vol 13 (10) ◽  
pp. e234865
Author(s):  
Nicholas Avdimiretz ◽  
Amy Glicksman ◽  
Sharon Dell ◽  
Philip John ◽  
Theo J Moraes
Keyword(s):  
Large Volume ◽  
Blood Clot ◽  
Short Term ◽  
Stepwise Approach ◽  
Symptom Resolution ◽  
Ct Angiogram ◽  
Intravenous Antibiotics ◽  
Endobronchial Lesion ◽  
Pulmonary Arterial ◽  
Multiple Episodes

A 9-year-old previously well girl presented with multiple episodes of large volume haemoptysis and right sided consolidation. She continued to have haemoptysis despite intravenous antibiotics. CT chest suggested a right mainstem endobronchial lesion; this was not seen on bronchoscopy where an extensive blood clot was removed. Distal flexible bronchoscopy could not identify the source of bleeding. CT angiogram revealed a broncho-pulmonary arterial fistula, a rare cause of haemoptysis in children. Endovascular embolisation resulted in short-term symptom resolution; however, haemoptysis recurred months later, leading to re-embolisation. This case highlights a stepwise approach to the workup of large volume haemoptysis.

scholarly journals Risk stratification in PAH

2020 ◽  
Vol 2020 (1) ◽  
Author(s):  
Paul A Corris
Keyword(s):  
Heart Failure ◽  
Pulmonary Arteries ◽  
Right Heart Failure ◽  
Response To Therapy ◽  
Pulmonary Vasculature ◽  
Short Term ◽  
Right Heart ◽  
Term Survival ◽  
Pulmonary Arterial ◽  
Short Term Survival

[No abstract. Showing first paragraph of article]Pulmonary arterial hypertension (PAH) is a chronic disease of the pulmonary vasculature characterized by progressive narrowing of the pulmonary arteries leading to increased pulmonary vascular resistance, right heart failure, and ultimately premature death.There has been a significant improvement in the available medical therapeutic options in this field that have impacted the short-term survival and morbidity in these patients. However, the median survival post-diagnosis remains unacceptable at 7 years.Physicians’ ability to predict PAH disease progression and risk allows them to determine the patient’s prognosis, make informed adjustments to therapy, and monitor his or her response to therapy . If widely adopted, risk prediction can enhance the consistency of treatment approaches and improve the timeliness of referral for lung transplantation. This approach should lead optimal, directed care that ultimately reduces morbidity and improves mortality in patients with PAH.

Doxycycline Injection Treatment for Unicameral Bone Cysts: a Case Series

2021 ◽  
Author(s):  
Andrew Albert ◽  
Monte Squiers ◽  
Eric E. Poole ◽  
Bennett W. Hartley ◽  
Maxwell V. Phillips ◽  
...  
Keyword(s):  
Side Effects ◽  
Matrix Metalloproteinases ◽  
Case Series ◽  
Pathologic Fracture ◽  
Bone Cysts ◽  
Short Term ◽  
Symptom Resolution ◽  
Osteoclast Activity ◽  
Injection Treatment

Abstract Background: Unicameral bone cysts (UBCs) are frequently associated with pathologic fracture due to aggressive osteolysis. Methods/Results: We present a case series (n=5) with complex or refractory bone cysts treated with doxycycline injections that exhibited increased ossification and symptom resolution at short term follow up with minimal side effects. Conclusions: In addition to its antibiotic properties, doxycycline is also known to inhibit matrix metalloproteinases, angiogenesis, and osteoclast activity suggesting that UBCs are dependent on MMP, VEGF or RANKL-mediated osteolysis. Further investigation is warranted regarding the use of doxycycline injections in UBCs.

scholarly journals Short-term mechanisms activated by the nerve growth factor NGF to induce pulmonary arterial hyperreactivity

2019 ◽  
Vol 11 (2) ◽  
pp. 202
Author(s):  
G. Cardouat ◽  
M. Douard ◽  
P. Robillard ◽  
M. Dubois ◽  
R. Marthan ◽  
...  
Keyword(s):  
Nerve Growth Factor ◽  
Growth Factor ◽  
Short Term ◽  
Nerve Growth ◽  
Pulmonary Arterial

scholarly journals Case report: Stepwise transition from subcutaneous treprostinil to epoprostenol in high-risk pulmonary arterial hypertension

2021 ◽  
Vol 5 (10) ◽  
Author(s):  
Ana Laura Mori ◽  
Andrea Rodríguez ◽  
Juan Alberto Gagliardi ◽  
Alejandro Stewart Harris
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
High Risk ◽  
Arterial Hypertension ◽  
Combined Treatment ◽  
Functional Class ◽  
High Morbidity ◽  
Stepwise Approach ◽  
Pulmonary Arterial ◽  
High Doses ◽  
Prostacyclin Analogues

Abstract Background Idiopathic pulmonary arterial hypertension is associated with high morbidity and mortality. In recent years, the use of targeted therapies has led to an improvement in prognosis. Prostacyclin analogues treprostinil and epoprostenol require continuous subcutaneous or intravenous infusion and are generally administered in a stepwise approach. However, there are no clear recommendations for transition in high-risk patients requiring high doses of prostacyclin analogues. Case summary In this report, we describe the case of a 20-year-old woman under combined treatment with sildenafil, macitentan, and treprostinil who required transition from subcutaneous treprostinil therapy to intravenous epoprostenol due to erratic drug absorption and functional class progression. The transition was performed over 48 h in a stepwise approach reducing treprostinil dose 4 ng/kg/min every 3 h while increasing epoprostenol infusion 2 ng/kg/min until achieving a maintenance dose of 32 ng/kg/min. There were no side effects requiring changes in the infusion rate. Discussion Patients with advanced pulmonary arterial hypertension may necessitate switching from subcutaneous treprostinil to epoprostenol. Although many protocols have been used to date, there are no guidelines to direct this process safely. This 48-h scheme based on the pharmacokinetic properties of each drug was successful and well-tolerated.

Surgeon-Performed Ultrasound for Chronic Obstructive Sialadenitis Helps Predict Sialendoscopic Findings and Outcomes

2017 ◽  
Vol 157 (6) ◽  
pp. 973-980 ◽  
Author(s):  
Andrew R. Larson ◽  
Annick Aubin-Pouliot ◽  
Elise Delagnes ◽  
Melissa Zheng ◽  
Jolie L. Chang ◽  
...  
Keyword(s):  
San Francisco ◽  
Predictive Value ◽  
Case Series ◽  
Chronic Obstructive ◽  
Salivary Duct ◽  
Short Term ◽  
Symptom Resolution ◽  
Symptom Response ◽  
Obstructive Sialadenitis ◽  
The University

Objective To determine the accuracy of surgeon-performed ultrasound (SP-US) features for predicting intraoperative sialendoscopic findings and to correlate ultrasound features with short-term symptomatic outcomes following sialendoscopy-assisted salivary duct surgery (SASDS). Study Design Case series with chart review. Setting The practices of 2 academic sialendoscopy surgeons. Subjects and Methods Between February 2014 and September 2015, 82 patients with 105 symptomatic glands underwent SASDS, of whom 56 patients (75 glands) met inclusion criteria. SP-US and sialendoscopic findings were reviewed for patients with chronic obstructive sialadenitis who underwent SASDS and who had been prospectively evaluated with the University of California–San Francisco Chronic Obstructive Sialadenitis Symptoms (COSS) questionnaire (scored 0-100) administered preoperatively and 3 months postoperatively. Results The positive predictive value (PPV) and negative predictive value (NPV) of SP-US for identifying sialoliths intraoperatively were 94% (33/35) and 91% (39/43), respectively. Of glands with sialoliths on SP-US, 97% (34/35) had complete symptom resolution (COSS ≤10) after SASDS. The PPV and NPV of salivary duct dilation on SP-US for finding a corresponding stenosis on sialendoscopy were 93% (14/15) and 50% (14/28), respectively. Nonsialolith SP-US features with the highest PPV for complete or partial symptom resolution (COSS ≤25) following SASDS included distal duct dilation of all glands (86%; 6/7) and dilation of both distal and proximal parotid ducts (75%; 6/8). Conclusion SP-US has high accuracy for ruling in and ruling out sialoliths, as well as for ruling in but not for necessarily ruling out stenosis presence and location. Ultrasound is helpful in estimating degree of short-term symptom response following SASDS.

Short-term Clinical Effects of Tolvaptan, an Oral Vasopressin Antagonist, in Patients Hospitalized for Pulmonary Arterial Hypertension

2011 ◽  
Vol 17 (9) ◽  
pp. S166-S167
Author(s):  
Junichi Oomura ◽  
Takeshi Arita ◽  
Masakiyo Nobuyoshi ◽  
Seiji Iwabuti ◽  
Masahiko Gouya ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Clinical Effects ◽  
Short Term ◽  
Vasopressin Antagonist ◽  
Pulmonary Arterial

Hypoxic pulmonary vasoconstriction and gas exchange in acute canine pulmonary embolism

1996 ◽  
Vol 80 (4) ◽  
pp. 1240-1248 ◽  
Author(s):  
M. Delcroix ◽  
C. Melot ◽  
F. Vermeulen ◽  
R. Naeije
Keyword(s):  
Pulmonary Embolism ◽  
Gas Exchange ◽  
Sulfur Hexafluoride ◽  
Blood Clot ◽  
Hypoxic Pulmonary Vasoconstriction ◽  
Autologous Blood ◽  
Response Curves ◽  
Pulmonary Hemodynamics ◽  
Pulmonary Vasoconstriction ◽  
Pulmonary Arterial

Hypoxic pulmonary vasoconstriction (HPV) is inhibited in several models of acute lung injury. Whether HPV is preserved in pulmonary embolism is unknown. We investigated the effects of a reduction in the fraction of inspired O2 (FIO2) on pulmonary hemodynamics and gas exchange in anesthetized dogs before and after autologous blood clot pulmonary embolism. In a first group of 14 dogs, stimulus-response curves for HPV were constructed as pulmonary arterial pressure (Ppa) vs. FIO2 varied between 1.0 and 0.06 at a cardiac output (Q) kept constant at 3.5 l.min-1.m-2. Gas exchange was evaluated by using the multiple inert-gas elimination technique at FIO2 of 1.0, 0.4, and 0.1. Embolism decreased the relative magnitude of HPV, expressed as the gradient between Ppa and pulmonary arterial occluded pressure in hypoxia divided by (Ppa-pulmonary arterial occluded pressure) at FIO2 of 1.0, from 1.8 to 1.2 (P < 0.05). Retention minus excretion gradients for sulfur hexafluoride and ethane were increased by decreased FIO2 (P < 0.005 and P < 0.05, respectively) before but not after embolism. Hypoxia-induced deterioration in gas exchange before embolism was related to the amount of baseline very low ventilation-perfusion (VA/Q) ratios. Similar results were obtained in a second group of seven dogs with Q decreased to maintain Ppa at the same average value as before embolism. However, gas exchange was not affected by inspiratory hypoxia before as well as after embolism in this group, which presented with a lesser amount of baseline very low VA/Q. In both groups of dogs, increase in the FIO2 from 0.4 to 1.0 did not affect gas exchange. We conclude that 1) pulmonary embolism is associated with a partial inhibition of HPV, 2) HPV does not contribute to preserve gas exchange in pulmonary embolism, and 3) a strong HPV may deteriorate gas exchange in severe hypoxia in the presence of minor very low VA/Q inequality.

scholarly journals Combining bosentan and sildenafil in pulmonary arterial hypertension patients failing monotherapy: real-world insights

2015 ◽  
Vol 46 (2) ◽  
pp. 414-421 ◽  
Author(s):  
Fabio Dardi ◽  
Alessandra Manes ◽  
Massimiliano Palazzini ◽  
Cristina Bachetti ◽  
Gaia Mazzanti ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Combination Therapy ◽  
Arterial Hypertension ◽  
Real World ◽  
Survival Rates ◽  
Short Term ◽  
Real World Setting ◽  
Sequential Combination ◽  
Pulmonary Arterial ◽  
Heritable Pulmonary Arterial Hypertension

Pulmonary arterial hypertension is a severe disease with a complex pathogenesis, for which combination therapy is an attractive option.This study aimed to assess the impact of sequential combination therapy on both short-term responses and long-term outcomes in a real-world setting.Patients with idiopathic/heritable pulmonary arterial hypertension, or pulmonary arterial hypertension associated with congenital heart disease or connective tissue disease and who were not meeting treatment goals on either first-line bosentan or sildenafil monotherapy, were given additional sildenafil or bosentan and assessed after 3–4 months. Double combination therapy significantly improved clinical and haemodynamic parameters, independent of aetiology or the order of drug administration. Significant improvements in functional class were observed in patients with idiopathic/heritable pulmonary arterial hypertension. The 1-, 3- and 5-year overall survival estimates were 91%, 69% and 59%, respectively. Patients with pulmonary arterial hypertension associated with connective tissue disease had significantly poorer survival rates compared to other aetiologies (p<0.003).The favourable short-term haemodynamic results and good survival rates, observed in patients receiving both bosentan and sildenafil, supports the use of sequential combination therapy in patients failing on monotherapy in a real-world setting.

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