Sistema Regional de Información
en línea para Revistas Científicas de América Latina,
el Caribe, España y Portugal

The adult-onset Still’s disease is an autoinflammatory polygenic rare systemic condition, nonetheless, clinically well described.  Because of its sporadic presentation in adults with a potential severe inflammatory start, linked to a broad spectrum of manifestations and complications, it presents as a non-solved challenge to clinicians, with limited therapeutic options. In this article a patient´s case of the San Juan de Dios Hospital will be reviewed. Afterwards, general aspects such as clinical presentation, diagnostic criteria, associated complications y and therapeutic options will be described.  

Diabetic cardiomyopathy is an entity that originates from a microvascular complication of diabetes mellitus type 2, which develops by the synergy of several metabolic elements generated by chronic hyperglycemia and oxidation of free fatty acids that cause a harmful impact by different biochemical pathways at the level of the myocardium causing structural and functional changes, as a consequence they generate diastolic and early systolic dysfunction before producing associated symptoms. Due to the above it is very important to understand this pathology from the physiopathological level, on the other hand, to establish an opportune diagnosis by established methods such as echocardiography and possible treatments at present for the improvement of myocardial contractility. In the present article we review the physiopathological mechanisms based on the metabolic processes in diabetes mellitus that affect the myocardium, generating a decrease in cardiac efficiency, thus causing a long-term heart failure, and also analyzing the most precise and conclusive diagnostic methods. to establish diabetic cardiomyopathy. On the other hand, the therapeutic effects of the inhibitory drugs of the sodium-glucose cotransporter type 2 in the myocardium will be analyzed on the basis of their benefits already proven benefits and in theories and hypotheses that have been raised from experimental and observational investigations.

Ectopic pregnancy (EP) or extra uterine pregnancy, accepted from the Greek word ‘‘ektopos’’ meaning out of place, refers to the blastocyst implantation outside the uterine cavity endometrium. The fallopian tube is the dominant site in the majority of cases of ectopic pregnancy. Approximately, 75% of deaths in the first trimester and 9% of all pregnancy-related deaths are due to EP. The etiology of EP is multifactorial. The postulated mechanism includes (1) anatomic obstruction, (2) abnormalities in tubal motility or ciliary function, (3) abnormal conceptus, and (4) chemotactic factors stimulating tubal implantation. The triad of pain, vaginal bleeding and amenorrhea was historically used to suspect a diagnosis of EP. The symptoms of EP are often non-specific and difficult to differentiate from those of other gynecological, gastrointestinal and urological disorders. The use of transvaginal scan (TVS) has changed the diagnostic approach to one based on visualizing the ectopic mass. TVS has been demonstrated to be superior to transabdominal ultrasound (TAS). Management options include expectant management, medical treatment, conservative surgery and radical surgery. With early diagnosis, most women with ectopic pregnancy could be treated with methotrexate (MTX). Currently, there are three main treatment regimens for management of ectopic pregnancy with methotrexate: the multidose protocol, the single-dose protocol, and the two-dose protocol.

The defects of the neural tube are serious congenital malformations whose prenatal diagnosis allows to guide the obstetric management, this can also increase the expectations of the users of the medical services and increase even more the existing complexity in the analysis of the causal link in forensic analysis of complaints about professional responsibility, and justifies the updating of concepts about the etiological risk factors of these defects, which are identified in the recent scientific medical literature. With this objective, this review is carried out, obtaining that the etiology of most neural tube defects seems to be multifactorial and involves genetic and environmental factors and probably requires the contribution of more than one element, so it is not possible to relate directly to a single factor, such as the lack of folic acid supplement during the prenatal period, with a malformation of this type, or rule out other causes, in addition to many of the molecular mechanisms are still under study. In establishing the causal link there must be sufficient evidence to fully accept that there is a direct relationship between non- exposure or exposure to a factor and the clinical effect (outcome). It is hoped that the discovery of new genes and epigenetic factors related to the defect will produce new preventive approaches; improved genetic counseling; and improved prognostic information and new therapeutic strategies, for which the forensic medical analysis will increase in complexity if complaints are established in this regard.

For this article, a bibliographic review of pemphigus has been made, with emphasis on the types vulgaris and foliaceus, using several databases such as: AAD, UptoDate, JAAD, NEJM, ScieLo, The Bmj, IntraMed, BINASSS, MedScape; with the objective of updating the information on this disease, in terms of: epidemiology, etiopathology, most frequent signs and symptoms, most commonly used diagnostic methods and treatment. It is an autoimmune dermatological disease, its main lesion is vesicular-bullous with a background that could be erythematous or not. In most of the investigations, there is talk of no predilection based on gender, however, there are other studies that contradict it. Its two main types are vulgaris and foliaceus, which will be discussed in this review. In Costa Rica, it is one of the autoimmune bullous diseases with the highest percentage of deaths. The diagnosis is based in the clinical findings as well as in the histologic and immunologic findings as confirmatory measures. The treatment consists mainly in systemic corticosteroids, which can be used in combination with immunosuppressants or other drugs such as rituximab, in order to reduce the dosage of corticosteroids and thus their common adverse effects.

Breast cancer is a frequent pathology at present. Studies report that a high proportion of patients develop lymphedema as a result of cancer or its treatment. Lymphedema can interfere with activities of daily living causing a restriction in range of motion, pain, increased skin tension, recurrent infection, edema of the extremities and the patient’s perception of the sensation of heaviness in the extremity, affecting their quality of life. Non-surgical options have traditionally been the mainstay and remain the initial form of treatment for all stages of lymphedema. Although effective, these therapies have several drawbacks. Naturally, doctors have sought other treatment modalities to improve the quality of life of the patient and reduce the economic burden of lymphedema. Several surgical interventions have been described for the treatment of lymphedema, any surgical intervention must be based on well-defined indications.

Spinal cord compression syndrome is one of the most frequent oncologic emergencies that physicians face in the clinical approach of the patient with cancer that curses with acute neurological deficit. Around this clinical entity has been established a debate about the more appropriate therapeutic approach; however retrospective studies have failed to established a clear protocol of treatment and the different therapeutic techniques haven´t shown any significant differences in prognosis in short and long term.

Acute Respiratory Syndrome (ARDS) is a form of non cardiogenic pulmonary edema, due to alveolar injury and it is diagnosed according to the Berlin definition. It is associated with a mortality of 20- 40% approximately. e ARDS is characterized by three sequential phases: the exudative, the proliferative and the fibrotic phase. It presents a wide differential diagnosis. Management is based in support as protective mechanical ventilation, prone position ventilation and the avoidance of fluid overload. e use of steroids, statins, nitric oxide and high-frequency oscillation ventilation do not have a clear benefit. Nowadays, strategies to prevent ARDS are on study.

Bouveret syndrome is a rare pathology that generates obstruction of the gastric outlet tract due to the passage of a large gallstone through a bilio-duodenal fistula. Later, there is impaction of the gallstone in the duodenum, which produces an intestinal obstruction. It is considered a rare variant of gallstone ileus that typically occurs in patients with advanced age and in association with biliary diseases. Because it is a little known syndrome, its diagnosis is often overlooked and adequate treatment is not provided to a patient who may have a high morbidity and mortality rate.

Takotsubo cardiomyopathy is a recently described entity (in 1990 in Japan). This pathology occurs mainly in postmenopausal women, and the greater incidence has been described during the summer. The pathophysiology of the disease is not completely clear at present; however, the catecholaminergic excess, triggered by physical or emotional stress and the subsequent inhibition of adrenergic receptors may be responsible. Takotsubo cardiomyopathy presents clinically indistinguishable from infarction, and is necessary the laboratory tests and imaging studies to perform the differential diagnosis. This disease present a good prognosis, but complications can occur