scholarly journals Osteopetrosis. Presentación de dos casos [Non-surgical management of osteopetrosis: two case reports an review of the literature]

Author(s):  
Juan Carlos Caruso ◽  
Juan Martín Patiño

Las osteopetrosis (enfermedad de Albers-Schönberg) es un síndrome con cuatro tipos clásicos e instituye una displasia ósea secundaria a la falta de resorción de hueso por anormalidad de los osteoclastos, lo cual provoca un tejido óseo duro y quebradizo,propenso a fracturas difíciles de tratar quirúrgicamente. Se han publicado escasos artículos sobre el tema; por este motivo, decidimos presentar a dos pacientes con fracturas diafisarias de húmero con osteopetrosis, ambas tratadas en forma incruenta.Los objetivos son comunicar nuestra experiencia y el método de tratamiento de dicha afección y realizar una revisión bibliográfica acerca del tema. Creemos que el tratamiento de elección para las fracturas diafisarias de húmero en pacientes con osteopetrosises el incruento, ya que su tipo de tejido óseo dificulta la implementación de cualquier osteosíntesis. Además, la colocación de implantes puede provocar algunas complicaciones, como infecciones, retraso de la consolidación y seudoartrosis. La cirugía se reserva para ciertos casos, como en pacientes con riesgo de desarrollar deformidades incapacitantes, aquellos que han sufrido fracturas repetidas, con retraso de la consolidación, seudoartrosis, quienes no responden al tratamiento incruento o con unadeformidad previa.AbstractOsteopetrosis (also known as Albers-Schönberg disease) is a syndrome that includes four classic types and is characterized by bone dysplasia and lack of bone resorption due to abnormal osteoclastic activity and consequent development of brittle and hard bone that is prone to fractures that are difficult to treat surgically. Herein we present two cases of osteopetrosis with diaphyseal fractures of the humerus, both managed with non-surgical treatment. The objectives of our manuscript are to document our experience in the management of these cases and review the literature. The non-invasive treatment provides the best outcome for dyaphyseal fractures on the humerus in patients with osteopetrosis, given that the quality of the bone in these patients impairs the implementation of osteosynthesis. In addition, the placement of implants can lead to complications such as infections, delayed consolidation and pseudoarthrosis, among others. Surgical treatment should reserved for certain patients such as those with delayed consolidation, pseudoarthrosis, a history of repeated fractures, pre-existing deformity and those who are at risk for the development of disabling deformities or do not respond to non-surgical treatment.

Cosmetics ◽  
2021 ◽  
Vol 8 (3) ◽  
pp. 56
Author(s):  
Tassahil Messas ◽  
Achraf Messas ◽  
George Kroumpouzos

Genitourinary syndrome of menopause (GSM) causes significant symptomatic aggravation that affects the quality of life (QoL). Vulvovaginal atrophy (VVA), the hallmark of GSM, is managed with topical non-hormonal therapy, including moisturizers and lubricants, and topical estrogen application. Patients not responding/being unsatisfied with previous local estrogen therapies are candidates for a noninvasive modality. Carbon dioxide (CO2) laser therapy, especially the fractionated type (FrCO2), has drawn considerable attention over the past two decades as a non-invasive treatment for GSM. This systematic review describes the accumulated evidence from 40 FrCO2 laser studies (3466 participants) in GSM/VVA. MEDLINE, Scopus and Cochrane databases were searched through April 2021. We analyze the effects of FrCO2 laser therapy on symptoms, sexual function, and QoL of patients with GSM/VVA. As shown in this review, FrCO2 laser therapy for GSM shows good efficacy and safety. This modality has the potential to advance female sexual wellness. Patient satisfaction was high in the studies included in this systematic review. However, there is a lack of level I evidence, and more randomized sham-controlled trials are required. Furthermore, several clinical questions, such as the number of sessions required that determine cost-effectiveness, should be addressed. Also, whether FrCO2 laser therapy may exert a synergistic effect with systemic and/or local hormonal/non-hormonal treatments, energy-based devices, and other modalities to treat GMS requires further investigation. Lastly, studies are required to compare FrCO2 laser therapy with other energy-based devices such as erbium:YAG laser and radiofrequency.


Diagnostics ◽  
2021 ◽  
Vol 11 (7) ◽  
pp. 1261
Author(s):  
Jin-Young Choi ◽  
Song-Soo Yang ◽  
Jong-Hwa Lee ◽  
Hyun-Jin Roh ◽  
Jun-Woo Ahn ◽  
...  

Background: Since the first report of a diaphragmatic hernia from Ambroise Paré’s necropsy in 1610, the Bochdalek hernia (BH) of the congenital diaphragmatic hernia (CDH) has been the most common types with high morbidity and mortality in the neonatal period. Due to the nature of the disease, CDH associated with pregnancy is too infrequent to warrant reporting in the literature. Mortality of obstruction or strangulation is mostly due to failure to diagnose symptoms early. Data sources and study selection: A systematic literature search of maternal BH during pregnancy was conducted using the electronic databases (PubMed and EMBASE) from January 1941 to December 2020. Because of the rarity of the disease, this review included all primary studies, including case reports or case series that reported at least one case of maternal BH in pregnant. Searches, paper selection, and data extraction were conducted in duplicate. The analysis was performed narratively regardless of the control groups’ presence due to their rarity. Results: The search retrieved 3450 papers, 94 of which were deemed eligible and led to a total of 43 cases. Results of treatment showed 16 cases in delayed delivery after hernia surgery, 10 cases in simultaneous delivery with hernia surgery, 3 cases in non-surgical treatment, and 14 cases in hernia surgery after delivery. Of 16 cases with delayed delivery after hernia surgery, 13 (81%) cases had emergency surgery and three (19%) cases had surgery after expectant management. Meanwhile, 10 cases underwent simultaneous delivery with hernia surgery, 6 cases (60%) had emergent surgery, and 4 cases (40%) had delayed hernia surgery after expectant management. 3 cases underwent non-surgical treatment. In this review, the maternal death rate and fetal/neonatal loss rate from maternal BH was 5% (2/43) and 16% (7/43), respectively. The preterm birth rate has been reported in 35% (15/43) of maternal BH, resulting from maternal deaths in 13% (2/15) of cases and 6 fetal loss in 40% (6/15) of cases; 44% (19/43) of cases demonstrated signs of bowel obstruction, ischemia, or perforation of strangulated viscera in the operative field, resulting from maternal deaths in 11% (2/19) of cases and fetal-neonatal loss in 21% (4/19) of cases. Conclusion: Early diagnosis and surgical intervention are imperative, as a gangrenous or non-viable bowel resection significantly increases mortality. Therefore, multidisciplinary care should be required in maternal BH during pregnancies that undergo surgically repair, and individualized care allow for optimal results for the mother and fetus.


2013 ◽  
Vol 2 (1) ◽  
pp. 1-12 ◽  
Author(s):  
Amanda Rice ◽  
Richard King ◽  
Evette Reed ◽  
Kimberley Patterson ◽  
Belinda Wurn ◽  
...  

Thorax ◽  
2019 ◽  
Vol 74 (9) ◽  
pp. 898-905 ◽  
Author(s):  
Elizabeth Sapey ◽  
Mona Bafadhel ◽  
Charlotte E Bolton ◽  
Thomas Wilkinson ◽  
John R Hurst ◽  
...  

In the nineteenth century, it was recognised that acute attacks of chronic bronchitis were harmful. 140 years later, it is clearer than ever that exacerbations of chronic obstructive pulmonary disease (ECOPD) are important events. They are associated with significant mortality, morbidity, a reduced quality of life and an increasing reliance on social care. ECOPD are common and are increasing in prevalence. Exacerbations beget exacerbations, with up to a quarter of in-patient episodes ending with readmission to hospital within 30 days. The healthcare costs are immense. Yet despite this, the tools available to diagnose and treat ECOPD are essentially unchanged, with the last new intervention (non-invasive ventilation) introduced over 25 years ago.An ECOPD is ‘an acute worsening of respiratory symptoms that results in additional therapy’. This symptom and healthcare utility-based definition does not describe pathology and is unable to differentiate from other causes of an acute deterioration in breathlessness with or without a cough and sputum. There is limited understanding of the host immune response during an acute event and no reliable and readily available means to identify aetiology or direct treatment at the point of care (POC). Corticosteroids, short acting bronchodilators with or without antibiotics have been the mainstay of treatment for over 30 years. This is in stark contrast to many other acute presentations of chronic illness, where specific biomarkers and mechanistic understanding has revolutionised care pathways. So why has progress been so slow in ECOPD? This review examines the history of diagnosing and treating ECOPD. It suggests that to move forward, there needs to be an acceptance that not all exacerbations are alike (just as not all COPD is alike) and that clinical presentation alone cannot identify aetiology or stratify treatment.


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