scholarly journals Congenital Chylothorax of the Newborn: A Systematic Analysis of Published Cases between 1990 and 2018

Respiration ◽  
2021 ◽  
pp. 1-13
Author(s):  
Bernhard Resch ◽  
Gülsen Sever Yildiz ◽  
Friedrich Reiterer

<b><i>Background:</i></b> Congenital chylothorax (CCT) of the newborn is a rare entity but the most common cause of pleural effusion in this age-group. We aimed to find the optimal treatment strategy. <b><i>Material and Methods:</i></b> A PubMed search was performed according to the PRISMA criteria. All cases were analyzed according to prenatal, perinatal, and postnatal treatment modalities and follow-ups. <b><i>Results:</i></b> We identified 753 cases from 157 studies published between 1990 and 2018. The all-cause mortality rate was 28%. Prematurity was present in 71%, male gender dominated 57%, mean gestational age was 34 weeks, and birth weight was 2,654 g. Seventy-nine percent of newborns had bilateral CCT, the most common associated congenital anomalies with CCT were pulmonary lymphangiectasia and pulmonary hypoplasia, and the most common chromosomal aberrations were Down, Noonan, and Turner syndromes, respectively. Mechanical ventilation was reported in 381 cases for mean 17 (range 1–120) days; pleural punctuations and drainages were performed in 32% and 64%, respectively. Forty-four percent received total parenteral nutrition (TPN) for mean 21 days, 46% medium-chain triglyceride (MCT) diet for mean 37 days, 20% octreotide, and 3% somatostatin; chemical pleurodesis was performed in 116 cases, and surgery was reported in 48 cases with a success rate of 69%. In 462 cases (68%), complete restitution was reported; in 34 of 44 cases (77%), intrauterine intervention was carried out. <b><i>Conclusion:</i></b> Respiratory support, pleural drainages, TPN, and MCT diet as octreotide remain to be the cornerstones of CCT management. Pleurodesis with OK-432 done prenatally and povidone-iodine postnatally might be discussed for use in life-threatening CCT.

2018 ◽  
Vol 2018 ◽  
pp. 1-10 ◽  
Author(s):  
Yasutaka Mitamura ◽  
Mika Murai ◽  
Chikage Mitoma ◽  
Masutaka Furue

Systemic fibrosing or sclerotic disorders are life-threatening, but only very limited treatment modalities are available for them. In recent years, periostin (POSTN), a major extracellular matrix component, was established by several studies as a novel key player in the progression of systemic fibrotic disease. In this research, we revealed the involvement of oxidative stress in the expression of POSTN induced by TGF-β1 and IL-13 in dermal fibroblasts. We found that the antioxidant cinnamaldehyde activated the NRF2/HMOX1 pathway. Cinnamaldehyde also alleviated TGF-β1- and IL-13-mediated production of reactive oxygen species and subsequent POSTN upregulation in dermal fibroblasts. In contrast, NRF2 silencing abolished the cinnamaldehyde-mediated downregulation of POSTN. These results suggest that cinnamaldehyde is a broad inhibitor of POSTN expression covering both TGF-β1 and IL-13 signaling. Cinnamaldehyde may thus be beneficial for the treatment of systemic fibrotic diseases.


Author(s):  
Toshiki Kuno ◽  
Yujiro Yokoyama ◽  
Alexandros Briasoulis ◽  
Makoto Mori ◽  
Masao Iwagami ◽  
...  

Background Although current guidelines recommend dual antiplatelet therapy (DAPT) for 3 to 6 months following transcatheter aortic valve replacement (TAVR), there are no studies directly comparing outcomes of different durations of DAPT following TAVR. Methods and Results PubMed, EMBASE, and Cochrane Database were searched through November 2020 to identify clinical studies that investigated single antiplatelet therapy versus DAPT use following TAVR. Studies using oral anticoagulants and antiplatelet therapy concomitantly were excluded. The DAPT group was subdivided by the duration of DAPT. We extracted the risk ratios (RRs) of major or life‐threatening bleeding, stroke, and all‐cause mortality. Four randomized controlled trials, 2 propensity‐score matched studies, and 1 observational study were identified, yielding a total of 2498 patients who underwent TAVR assigned to the single antiplatelet therapy group (n=1249), 3‐month DAPT group (n=485), or 6‐month DAPT group (n=764). Pooled analyses demonstrated that when compared with the single antiplatelet therapy group, the rates of major or life‐threatening bleeding were significantly higher in the 3‐ and 6‐month DAPT groups (RR [95% CI]=2.13 [1.33–3.40], P =0.016; RR [95% CI]=2.54 [1.49–4.33], P =0.007, respectively) with no difference between the 3‐month DAPT versus 6‐month DAPT groups. The rates of stroke and all‐cause mortality were similar among the 3 groups. Conclusions In this network meta‐analysis of antiplatelet therapy following TAVR, single antiplatelet therapy with aspirin had lower bleeding without increasing stroke or death when compared with either 3‐ or 6‐month DAPT.


Author(s):  
David E. Krummen ◽  
Gordon Ho ◽  
Kurt S. Hoffmayer ◽  
Franz Schweis ◽  
Tina Baykaner ◽  
...  

Background - Refractory ventricular fibrillation (VF) is a challenging clinical entity, for which ablation of triggering premature ventricular complexes (PVCs) is described. When PVCs are infrequent and multifocal, the optimal treatment strategy is uncertain. Methods - We prospectively enrolled consecutive patients presenting with multiple ICD shocks for VF refractory to antiarrhythmic drug therapy, exhibiting infrequent (≤3%), multifocal PVCs (≥3 morphologies). Procedurally, VF was induced with rapid pacing and mapped, identifying sites of conduction slowing and rotation or rapid focal activation. VF electrical substrate ablation (VESA) was then performed. Outcomes were compared against reference patients with VF who were unable or unwilling to undergo catheter ablation. The primary outcome was a composite of ICD shock, electrical storm, or all-cause mortality. Results - VF was induced and mapped in 6 patients (60±10 y, LVEF 46±19%) with ischemic (n=3) and nonischemic cardiomyopathy. An average of 3.3±0.5 sites of localized reentry during VF were targeted for radiofrequency ablation (38.3±10.9 minutes) during sinus rhythm, rendering VF non-inducible with pacing. Freedom from the primary outcome was 83% in the VF ablation group versus 17% in 6 non-ablation reference patients at a median of 1.0 years (IQR 0.5-1.5 years, p=0.046) follow-up. Conclusions - VESA is associated with a reduction in the combined endpoint compared with the non-ablation reference group. Additional work is required to understand the precise pathophysiologic changes which promote VF in order to improve preventative and therapeutic strategies.


2021 ◽  
Vol 9 ◽  
Author(s):  
Hung-Lin Tai ◽  
Tze Yee Diane Mok ◽  
An-Shine Chao ◽  
Shih-Ming Chu ◽  
Reyin Lien

Background: Idiopathic congenital chylothorax is a rare but serious disease. Advancement in perinatal care and the renovated treatment modalities have brought about significant improvement in patient outcome.Objective: To describe the clinical course of severe forms of idiopathic congenital chylothorax, focusing on the development of recent treatment modalities and their impacts.Design: A retrospective cohort by review of medical records in the NICU of a perinatal referral center in Taiwan. Study period was from January 2006 to June 2017. Neonates with the diagnosis of idiopathic congenital chylothorax with non-immune hydrops fetalis were enrolled. Clinical relevant including demographic data, perinatal interventions, post-natal course, and treatment outcome were described and analyzed.Results: Twenty-eight neonatal patients were included. The median gestational age at birth was 34 (range 27–36) weeks and median birth weight was 2,369 (range 1,430–3,608) g. Prenatal intervention was performed in 39.3% of the patients. Besides, 11 out of the 28 neonates developed tension pneumothorax in the first 24 h and 4 (36.4%) of them died. Sepsis was documented in two patients (7.1%). Overall survival rate was 71.4%. There were five patients enrolled during the last 2 years of the study period. EXIT with intubation was performed in two and octreotide was given to four of these most recent neonates, and all of them survived.Conclusion: Recent advances in the management of these neonates, specifically EXIT with intubation and use of octreotide. Both of them improved patient survival in our cohort, but the evidence of impact has yet to be validated.


2018 ◽  
Vol 22 (6) ◽  
pp. 591-601 ◽  
Author(s):  
Linghong Linda Zhou ◽  
Jorge R. Georgakopoulos ◽  
Arvin Ighani ◽  
Jensen Yeung

Generalized pustular psoriasis (GPP) is a rare but serious and difficult to treat cutaneous disease, with high morbidity and mortality rates. Despite the numerous treatment regimens available, the overall quality of evidence-based research is limited with a lack of an algorithmic approach available. In this review, we aim to evaluate the current level of evidence regarding the efficacy and safety/tolerability of systemic monotherapies available in the treatment of GPP. A comprehensive MEDLINE, EMBASE, and PubMed search of clinical studies examining systemic monotherapy treatment options for GPP was conducted. In total, 31 studies met eligibility criteria. Described treatment modalities included retinoids, cyclosporine, biologics, and dapsone. Despite the lack of high-quality evidence or a well-accepted treatment algorithm for GPP, systemic retinoids, cyclosporine, biologics, and dapsone are all possible first-line agents, with retinoids being one of the best-supported treatment options and biologics as an emerging therapeutic field with great potential requiring additional data. However, the final choice of treatment should be considered within the unique context of each patient.


2021 ◽  
Author(s):  
Marie-Kristin Schwaegermann ◽  
Lukas Hobohm ◽  
Johanna Rausch ◽  
Michael Reuter ◽  
Thomas-Friedrich Griemert ◽  
...  

AbstractImmune thrombotic thrombocytopenic purpura (iTTP) is a rare autoimmune disorder characterized by severely reduced activity of the von Willebrand factor (VWF)-cleaving protease ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) due to autoantibodies. This leads to the development of pathogenic multimers of VWF, causing a thrombotic microangiopathy with decreased number of platelets, hemolysis, and life-threatening tissue ischemia of mostly brain, heart, and kidneys. Standard treatment of iTTP involves daily plasma exchange to remove ultra large multimers of VWF, inhibitors, substituting ADAMTS13, and the accompaniment of an immunosuppressive treatment with steroids. Recently, caplacizumab was approved for iTTP. Caplacizumab is a nanobody binding the A1 domain of VWF, blocking its interaction with glycoprotein Ib–IX–V platelet receptor and therefore preventing platelet aggregation. VWF activities may serve as therapeutic drug monitoring of caplacizumab, whereas ADAMTS13 activities may be used for biomarkers to guide caplacizumab treatment modalities and overall treatment duration. Additional immunosuppressive treatment by inhibiting autoantibody formation (e.g., the use of Rituximab, a chimeric monoclonal antibody directed against the B-cell antigen CD20) is a further treatment option. Infections are well-known causes for an acute episode for patients with iTTP. The novel SARS-CoV-2 virus is mainly associated with acute respiratory distress as well as diffuse endothelial inflammation and increased coagulopathy. However, little is known about an infection with SARS-CoV-2 virus triggering iTTP relapses. We herein report the case of an acute iTTP episode accompanying a SARS-CoV-2 infection.


Author(s):  
Poonam Bharat Mandhare ◽  
Deepa R. Kale

Background: Our health and overall well-being mainly depends on our eating habits, physical activities, sleep patterns etc. Human beings, in order to adjust themselves in the modern era, have been compiled to become fast and mechanical. Due to this they can’t give proper attention to diet and exercise. Due to this Aaharpachan Kriya gets affected leading to Agnimandya (low or decreased digestive power). According to Ayurveda Agnimandya is responsible for almost all diseases. Objectives: Objective is to study importance of Takrasevan in Agnimandya.  Material and methods: Ayurvedic / modern texts and literature, research paper. Discussion: Grahani, Atisar, Arsh etc. are the commonest diseases seen in today’s life.  These diseases are not life threatening but can cause great distress to patient.  Ayurveda gave an amount of treatment modalities; one of them is Takrasevan i. e. consumption of buttermilk. Takra having ushna virya, kashaya rasa, madhur vipak and ruksha guna properties which pacifies Vata, Pitta, Kapha and potentiate the Agni and digests as well as absorbs the food articles and treats the disease in generous way. Conclusion: By this study it can be conclude that Takra (Buttermilk) can use on daily basis as dietary product. As it contains Probiotics which facilitates proper digestion and absorption process, it maintains proper metabolism to keep person free from diseases.   


1992 ◽  
Vol 3 (1) ◽  
pp. 180-189 ◽  
Author(s):  
Joanne Hughes Morscher

Wolff-Parkinson-White (WPW) syndrome is a cardiac conduction disorder that presents with potentially life-threatening consequences. Wolff-Parkinson-White syndrome-induced dysrhythmias account for 20% of all supraventricular tachycardias that occur in the general population. Clinical presentations range from no symptoms to a sudden cardiac arrest. The risk of sudden death is always present with WPW syndrome, and it is the motivating force in the evaluation and treatment of this syndrome. Current diagnostic modalities are accurate in identifying patients with WPW syndrome, but lack the sensitivity to predict sudden cardiac death. This article reviews the history of WPW syndrome, as well as its general characteristics, diagnostic criteria, treatment modalities, and nursing implications


Pharmacy ◽  
2019 ◽  
Vol 7 (3) ◽  
pp. 104 ◽  
Author(s):  
Leslie A. Hamilton ◽  
Anthony J. Guarascio

Despite the widespread use of tetracycline antibiotics since the late 1940s, tetracycline hypersensitivity reactions have rarely been described in the literature. A comprehensive PubMed search was performed, including allergic and serious adverse reactions attributed to the tetracyclines class of antibiotics. Of the evaluated tetracycline analogs, minocycline was attributed to the greatest overall number and severity of serious adverse events reported in the literature, with notable reactions primarily reported as respiratory and dermatologic in nature. Reactions to tetracycline have also been well described in the literature, and although dermatologic reactions are typically less severe in comparison with minocycline and doxycycline, various reports of anaphylactic reactions exist. Although doxycycline has been noted to have had the fewest reports of severe allergic reactions, rare descriptions of life-threatening reactions are still reported in the literature. Allergic reactions regarding tetracyclines are rare; however, adverse reaction type, severity, and frequency among different tetracycline analogs is somewhat variable. A consideration of hypersensitivity and adverse reaction incidence should be performed prior to the selection of individual tetracycline entities.


2005 ◽  
Vol 25 (4_suppl) ◽  
pp. 77-82 ◽  
Author(s):  
Akira Saito

Encapsulating peritoneal sclerosis (EPS) is a life-threatening complication of peritoneal dialysis (PD). The overall prevalence of EPS in Japanese PD patients is 2.3%. Among patients on PD for less than 5 years, the rate is 0.9%; among patients on PD for 5 – 10 years, the rate is 3.8%; and among patients on PD for >10 years, it is 11.5%. Thus, the longer the treatment duration, the higher the prevalence of EPS. Encapsulating peritoneal sclerosis does not result solely from the natural progression of peritoneal sclerosis. A “second hit” event, such as bacterial peritonitis, abdominal bleeding, or abdominal surgery may be needed to trigger the onset of EPS in the face of advanced peritoneal sclerosis. To prevent development of EPS, PD treatment is replaced by other treatments when patients reached high-transport status. Peritoneal lavage and prednisolone administration have been reported to be effective in preventing or stopping the progress of EPS. When bowel obstruction has occurred, total enterolysis to remove the fibrous capsule from the bowel is indicated. To maximize overall quality of life, patients with end-stage renal disease (ESRD) should have the choice to make use of all the treatment modalities available: PD, hemodialysis (HD), and transplantation. Furthermore, the development of truly biocompatible PD equipment—including peritoneal catheters, solutions, and systems—are desirable to extend PD treatment for the long term. The cost of individual products could decrease significantly if PD use were to increase to 30% from 10% among ESRD patients worldwide. As practitioners, we have to further improve the technical survival rate and functional duration of PD treatment so that adequate peritoneal function can be maintained for 10 years in at least 40% of PD patients. The goal is to place PD on par with HD using high-flux dialysis membranes and ultrapure dialysis solution.


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