159 research outputs found
The Acute Porphyric Attack: A Difficult Diagnosis for a Potential Lethal Event in Emergency Medicine
No full textThe porphyrias are a heterogeneous group of metabolic
disorders due to an inherited (but in some forms the disturbance may
also be acquired) enzymatic deficiency in the metabolic pathway
of heme biosynthesis. The variable degree of block in the heme
biosynthetic pathway due to the enzyme deficiency results in
accumulation of different metabolic intermediates, whose toxicity is
responsible for the peculiar (cutaneous and/or neurovisceral) clinical
pictures observed in each of these diseases. According to the clinical
features, the porphyrias are classified as “acute” (or neuropsychic)
[characterized by acute neurovisceral crises (the acute porphyric
attack) involving the autonomic and/or central nervous system, but
also the liver and the kidney] and “on acute” (or dermatological)
(mostly presenting with cutaneous lesions, due to photosensitivity).
The acute porphyrias are often misdiagnosed diseases: the acute
porphyric attack may in fact mimic many other more common medical
and neuropsychiatric conditions; its delayed diagnosis and treatment
(or its inappropriate treatment) may result in a fatal outcome. For
these reasons, many different specialists, such as surgeons,
psychiatrists, gastroenterologists, neurologists, emergency
physicians and dermatologists may be variably involved in the
diagnostic process, especially in those cases presenting with
acute and life-threatening clinical features. An early and definitive
diagnosis is mandatory to improve outcomes and to assure that
potentially harmful drugs are avoided. To date, the availability of an
adequate treatment has significantly improved the outcome of the
acute porphyric attacks, so the knowledge about the management of
these events may be relevant for the physicians working in internal
and emergency medicine units
Art in Science: The Artist and The Disease: The Exemplary Cases of Renoir and Toulouse-Lautrec
No full textFrom the Column Editors,
Our colleagues in Bologna and Milan, Italy have skillfully illustrated how physical illness shaped the artistry of two French 19thcentury icons, Pierre-Auguste Renoir and Henri de Toulouse-Lautrec. In the case of Renoir, he accommodated for his debilitating deformity and pain from arthritis through the use of technical contrivances that allowed him to manage canvases, access palettes, and grip brushes. These contraptions helped Renoir continue his prolific and creative career well beyond usual endurance. Renoir persisted in defining through color and brush stroke the inner light of Impressionism. For Toulose-Lautrec, his physical deformities drove him to seek asylum and anonymity at the margins of a Bohemian lifestyle found in Parisian night clubs. There, he found and painted the vulnerable characters this environment attracted and the theater they represented. For these insights, and for two of many examples of illness influencing art, we are indebted to Dr. Di Matteo and his colleagues
Platelet-rich plasma: evidence for the treatment of patellar and Achilles tendinopathy-a systematic review
No full textPlatelet-rich plasma (PRP) has been introduced in the clinical practice to treat a growing number of different musculoskeletal pathologies. It is currently applied in the treatment of Achilles and patellar tendinopathies, which are common sport-related injuries very challenging to manage. Aim of the present paper was to review systematically the available clinical evidence concerning the application of PRP in the treatment of patellar and Achilles tendinopathy. A systematic review of the literature was performed according to the following inclusion criteria for relevant articles: (1) clinical reports of any level of evidence, (2) written in the English language, (3) with no time limitation and (4) on the use of PRP to treat conservatively Achilles and patellar tendinopathy. Twenty-two studies were included and analyzed. Two studies on patellar tendinopathy were randomized controlled trials (RCTs), whereas just one RCT was published on Achilles tendon. All the papers concerning patellar tendon reported positive outcome for PRP, which proved to be superior to other traditional approaches such as shock-wave therapy and dry needling. In the case of Achilles tendon, despite the encouraging findings reported by case series, the only RCT available showed no significant clinical difference between PRP and saline solution. The main finding of this study was the paucity of high-level literature regarding the application of PRP in the management of patellar and Achilles tendinopathy. However, the clinical data currently available, although not univocal, suggest considering PRP as a therapeutic option for recalcitrant patellar and Achilles tendinopathies
Mechanisms of Neuronal Damage in Acute Hepatic Porphyrias
No full textPorphyrias are a group of congenital and acquired diseases caused by an enzymatic
impairment in the biosynthesis of heme. Depending on the specific enzyme involved, different types
of porphyrias (i.e., chronic vs. acute, cutaneous vs. neurovisceral, hepatic vs. erythropoietic) are
described, with different clinical presentations. Acute hepatic porphyrias (AHPs) are characterized
by life-threatening acute neuro-visceral crises (acute porphyric attacks, APAs), featuring a wide
range of neuropathic (central, peripheral, autonomic) manifestations. APAs are usually unleashed by
external “porphyrinogenic” triggers, which are thought to cause an increased metabolic demand for
heme. During APAs, the heme precursors -aminolevulinic acid (ALA) and porphobilinogen (PBG)
accumulate in the bloodstream and urine. Even though several hypotheses have been developed to
explain the protean clinical picture of APAs, the exact mechanism of neuronal damage in AHPs is
still a matter of debate. In recent decades, a role has been proposed for oxidative damage caused by
ALA, mitochondrial and synaptic ALA toxicity, dysfunction induced by relative heme deficiency on
cytochromes and other hemeproteins (i.e., nitric oxide synthases), pyridoxal phosphate functional
deficiency, derangements in the metabolic pathways of tryptophan, and other factors. Since the
pathway leading to the biosynthesis of heme is inscribed into a complex network of interactions,
which also includes some fundamental processes of basal metabolism, a disruption in any of the steps
of this pathway is likely to have multiple pathogenic effects. Here, we aim to provide a comprehensive
review of the current evidence regarding the mechanisms of neuronal damage in AHPs
PRP for the treatment of cartilage pathology.
No full textIn recent years biological strategies are being more widely used to treat cartilage lesions. One of the most exploited novel treatments is Platelet-rich Plasma (PRP), whose high content of growth factors is supposed to determine a regenerative stimulus to cartilaginous tissue. Despite many promising in vitro and in vivo studies, when discussing clinical application a clear indication for the use of PRP cannot be assessed. There are initial encouraging clinical data, but only a few randomized controlled trials have been published, so it is not possible to fully endorse this kind of approach for the treatment of cartilage pathology. Furthermore, study comparison is very difficult due to the great variability in PRP preparation methods, cell content and concentration, storage modalities, activation methods and even application protocols. These factors partially explain the lack of high quality controlled trials up to now. This paper discusses the main aspects concerning the basic biology of PRP, the principal sources of variability, and summarizes the available literature on PRP use, both in surgical and conservative treatments. Based on current evidence, PRP treatment should only be indicated for low-grade cartilage degeneration and in case of failure of more traditional conservative approaches
Rediscovering the history of orthopedics.
No full textHistory is difficult to digest, particularly for physicians like
us who are constantly pressed to deliver results, always
committed to the everyday medical practice, and required
to keep up to date with the most recent research. It is not
surprising, then, that in recent years there has been a
shortage of historical insight into indexed journals: where
can we find the time to read papers not dealing with the
progress of research or the latest updates in surgical procedures?
Therefore, why care about finding space to publish
manuscripts with different prerogatives
PRP injections versus viscosupplementation for early knee osteoarthritis: a randomized double-blind study
No full textObjectives: The influence of growth factors (GFs) on cartilage repair is not yet widely studied and its application in clinics is still experimental.
Platelet Rich Plasma (PRP), a blood product rich in GF, is a promising support for treating cartilage defects. Aim of this study is to evaluate and compare the efficacy of PRP and Viscosupplementation (Hyaluronic Acid—HA) injections for the treatment of chondropathy or early osteoarthritis (OA) of the knee.
Methods: 150 patients were enrolled: 72 were evaluated at 6 months follow-up. The study involved patients affected by chondropathy or early OA. All patients underwent an autologous blood harvesting, then 36 patients were randomized in the PRP group and 36 in the HA group. A cycle of 3 weekly injections was administered blindly. All patients were clinically evaluated at the enrolment, 2 months after the treatment and at 6 months follow up. IKDC, EQVAS, TEGNER and KOOS scores were used to clinically evaluate the patients. Adverse events and patient satisfaction were also recorded.
Results: No complications like infection, marked muscle atrophy, deep vein thrombosis, fever, haematoma, tissue hypertrophy, adhesion formation or other major adverse events occurred among study subjects. Only minor adverse events were detected in some patients, as mild pain reaction and effusion after the injections, in particular in the PRP group, but they lasted for no more than a few days. At the follow-up evaluations, both groups showed a significant improvement in terms of function and quality of life. The preliminary comparison between the outcomes of the two groups showed a not statistically significant difference, but with a trend slightly favourable for the PRP group at 6 months.
Conclusions: PRP is a simple, low cost and minimally invasive approach to treat chondropathy and early OA. The clinical results of our study are encouraging and suggest this method may be used to treat the degenerative articular pathology of the knee, leading to results at least comparable with those of HA. Long-term results will confirm the reliability and evaluate the durability of this promising procedure
Platelet rich plasma: a valid augmentation for cartilage scaffolds? A systematic review.
No full textIt has been shown that modern regenerative scaffold-based procedures for the treatment of articular cartilage defects offer good clinical results, although the properties of native healthy cartilage have not yet been matched by any substitute. Several implants have been tested and clinically used over the years to promote articular surface restoration, some of them producing a hyaline-like reparative tissue. There has been an increase in the number of new biological strategies, alone and in combination with scaffolds, to enhance the clinical outcome in patients with chondral disease. Among these innovative methods, one of the most widely used is Platelet-rich Plasma (PRP), based on the rationale of using the growth factors contained in platelet alpha granules to promote tissue regeneration. The aim of the present manuscript is to review systematically the current evidence in pre-clinical and clinical studies for the use of PRP augmented scaffolds to treat chondral or osteochondral disorders
Single-plug autologous osteochondral transplantation: results at minimum 16 years' follow-up.
No full textDifferent techniques have been proposed for the treatment of cartilage defects. Among the currently available options, autologous single-plug osteochondral transplantation is one of the few to be applied to address small and medium lesions. The goal of the current study was to document the long-term clinical outcome of a cohort of patients treated by this surgical strategy, which consists of harvesting a single osteochondral plug from a less weight bearing area of the knee and implanting it on the defect site by press-fit technique. Fifteen patients were enrolled. Age at surgery was 30.2±15.3 years, and body mass index was 22.5±3.0 kg/m(2). The inclusion criteria were clinical symptoms, such as knee pain or swelling, and grade III to IV chondral and osteochondral knee lesions. Patients were prospectively evaluated up to a mean of 17.5±3.5 years of follow-up by using Lysholm, International Knee Documentation Committee (IKDC) subjective, and Tegner scores. A significant improvement was noted in all clinical scores. In particular, the IKDC subjective score increased from 34.5±23.6 to 66.3±26.4 (P=.001). The Lysholm score showed a similar trend. From a baseline value of 47.8±29.5, the score increased to 79.8±24.6 at the last evaluation (P=.001). A significant increase in Tegner score was observed at the 2-year evaluation, with stable results up to the last follow-up. Four failures were reported, which in 3 cases occurred at mid- to long-term follow-up, confirming that this technique can be considered a suitable option for the treatment of small and medium chondral and osteochondral lesions in young patients
Knee scoring System
No full textKnee injuries are among the most common clinical conditions treated everyday by orthopaedic clinicians and sport medicine doctors. A single joint can be affected by a large variety of pathology, ranging from ligament lesions to cartilage defects and many others. As a consequence, this is a field of intense basic and clinical research, and there is a growing interest in better understanding how to evaluate knee condition at the moment of injury and after conservative or surgical treatments. Therefore it has been observed a great interest over time in developing clinical scores in order to make correlations with objective findings and give the clinician a suitable instrument for the assessment of the therapy outcome.
In the past years it has been observed a progressive trend from clinician-based outcome tools to patient-reported outcome measures.
The necessity of assessing the knee condition in the most effective way possible is one of the peculiar aspects justifying this growing interest; moreover, it must be also considered the drastic increase in patient consciousness about health and expected outcome after therapies and lastly, the interest by health insurers to better evaluate the expense concerning knee injuries and their recovery1.
These outcome evaluation instruments are patient-oriented2, strenghtening this way the importance of patient perception, which must be considered prevalent over the mere surgical result. Some studies have proved that patient satisfaction is strictly linked to outcome scores concerning subjective symptoms and function3. On the opposite side, some clinicians are not sure about the validity of this patient-reported tests, arguing that their subjective nature is a weak point, especially if compared to the more objective clinician-based findings4,5; even in this case, recent studies have proved that these clinical scores are often superior than the aforementioned clinical “objective” measures6-10.
In this chapter we will discuss in detail the scores employed in clinical studies focusing on knee treatments, in order to help the orthopedic surgeon in the choice of the most accurate evaluation tool according to the specific procedure performed
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