Marfan’s syndrome (MFS) is an autosomal dominant disorder that affects the body’s connective tissue, characterized by changes in three major connective tissue systems: the musculoskeletal, ocular, and cardiovascular systems. Individuals with MFS can develop severe cardiovascular complications., and the life expectancy of patients with MFS is markedly reduced as the effects of the disease on the aorta often lead to progressive dilatation, with subsequent dissection and rupture. Obstructive sleep apnea (OSA) is highly prevalent in patients with cardiovascular diseases. There is a high prevalence of OSA in patients with MFS due to craniofacial dysmorphism and increased upper airway collapsibility. A high prevalence of OSA has been reported in patients with MFS. To date, however, there are only a few case reports regarding the treatment of OSA in MFS patients. We present a treatment in an adult MFS patient with OSA using an oral appliance (OA). The patient was a 48-year-old Japanese woman of MFS, diagnosed with moderate OSA (apnea hypopnea index [AHI] = 27.0; lowest oxygen saturation [SaO2] = 78%; maximum apnea time = 114 s]). An OA was selected as the treatment option, and post-treatment sleep study indicated a reduction in AHI to 8.8/h with an improvement in other factors (lowest SaO2 = 84%; maximum apnea time = 66 s). Additionally, the treatment resulted in a reduction in BNP levels (81.3 from 110.2 pg/ml), and the patient’s cardiac function was stable over the period in which the patient received OA treatment. This study provides a basis for the future appropriate understanding and management of OSA of MFS patients.
Early effectivity of teduglutide for the treatment of short bowel sydrome – our first clinical experience
Background Short bowel syndrome is an uncommon malabsorptive condition most frequently caused by massive surgical resection of the small intestine. Standard of care is home parenteral nutrition. Teduglutide, glucagon like peptide 2 analog, was approved to treat patients with short bowel syndrome, who are stable following a period of post-surgery intestinal adaptation. Case presentation We report two cases of patients with short bowel syndrome on home parenteral nutrition, treated with teduglutide, novel advanced therapy. We monitored the effectiveness and tolerance of the new treatment. Conclusion Teduglutide increases intestinal absorption, causes cryptal hyperplasia, villous hypertrophy, angiogenesis, and allows weaning from parenteral nutrition. We confirmed the early onset of treatment effectiveness in the 4th month. Early effectiveness of treatment allowed a reduction in the volume of home parenteral nutrition with an improvement in quality of life.
BACKGROUND AND AIM: Adult, non-infective, haemolytic-uremic syndrome (HUS) although a rare disease in itself, has a high likelihood of occurrence in pregnancy and immediate post partum period. It is an important differential diagnosis in the evaluation of thrombotic microangiopathies. Patients with post-partum HUS display a classical triad of microangiopathic haemolytic anaemia, acute nephropathy and thrombocytopenia. I hereby present a case of post partum HUS treated with therapeutic plasma exchange (TPE) MATERIAL AND METHODS: A total of six sessions of TPE were performed daily, three sessions for consecutive days and remaining three sessions were performed on alternate days. All the procedures were carried out with Haemonetics MCS+ exchanging one plasma volume using fresh frozen plasma and saline as replacement fluid. Haemodialysis was started and four sessions were carried out on alternate days. RESULT: A 37 year old, 85 kg female, G2 P1, underwent emergency LSCS because of foetal distress at 38 weeks of pregnancy. Post surgery she developed decreasing urine output, anuria ensued. Emergency therapeutic plasma exchange was carried out within 24 hours of diagnosis. It could be found that with TPE, patient had improvement in renal function, decrease in LDH levels and increase in platelet count. Patient had sustained remission and discontinuation of haemodialysis. CONCLUSION: HUS is a disorder with high mortality and long term morbidity, if prompt treatment is not instituted. The decision to intervene with plasma exchange should be based upon the severity of thrombocytopenia, microangiopathic haemolytic anaemia and neurological abnormalities, even if the diagnosis and nomenclature is uncertain. Improved survival after this disorder has been attributed to aggressive treatment with plasma exchange therapy.
IMPACTS OF IMMUNOSUPPRESSION ON THE GRAVITY OF COVID-19 IN INDIVIDUALS CO-INFECTED WITH THE HUMAN IMMUNODEFICIENCY VIRUS (HIV)
Objective: To identify the levels of severity of COVID-19 in individuals co-infected with the Human Immunodeficiency Virus (HIV). Methods: Expanded summary of the literature review type. The searches were carried out in the main databases of the BVS and CAPES. The above authors declare that they do not have conflict of interest in this study. Results: Most individuals were being treated with antiretrovirals (83-100%) and, therefore, most had a TCD4 lymphocyte count above 200 cells/µL and a viral load ranging between 67-100%. Comorbidities ranged from 33-70% and deaths from 0-14%. The severity picture was mostly mild, at a minimum proportion of 63%, not significantly differing from the population with COVID-19 in general, which on average remains in a mild symptomatic picture, around 80%. Therefore, the percentage of deaths and more severe levels of the disease were lower than expected due to the immune deficiency of HIV positive people. Conclusion: It is rash to make generalizations about the topic or to safely close the hypothesis that the immunosuppression of individuals with HIV, co-infected with SARS-CoV-2 has an increased risk to acquire the most severe form of COVID-19. This is because the studies found in the literature did not have a sample of participants significant enough.
PARKINSONIA ACULEATA AS A POTENTIAL PHYTOTHERAPIC FACTOR IN THE INFLAMMATORY AND DYSLIPIDEMIC CONTROL OF WISTAR RATS
Objective: The study evaluated the hypolipidemic and anti-inflammatory potential of the hydroalcoholic extract of P. Aculeata (HEPa/EtOAC) in Wistar rats fed with Westernized diet. Methods: The experiment was approved by the Ethics Committee (Federal University of Pernambuco-23076.027165 / 2010-21). The hydroalcoholic extract (HEPa/EtOAc) was obtained from the aerial parts of P. Aculeata and portioned by acetate/ethanol in different concentrations. 36 male Wistar rats were initially divided into two groups: Control (C, n = 6), fed a commercial diet (26g / 100g of protein; 63g / 100g of carbohydrates; 11g / 100g of lipids) and Westernized (W; n = 30) – fed an experimental diet (15g / 100g of protein; 51g / 100g of carbohydrates; 34 g/100g of lipids) receving water ad libitum. After 4 months of diet, W group was arbitrarily redistributed into six subgroups according to water supplementation, or different extracts concentrations: (n = 6 animals/group): W- distilled water (1mL/kg; po); WG – genfibrozil (140 mg/kg; p.o.); W35- 35 mg/kg; W70 – 70 mg /kg; p.o. and W140- 140 mg/kg; p.o for 30 days. The lipid profile and pro-inflammatory cytokines were analyzed. Results: The westernized diet caused dyslipidemia and the treatment with HEPa-EtOAc (140mg / kg), promoted a significant reduction in the concentration of pro-inflammatory cytokines, in total cholesterol, in the LDLc and serum triglycerides. Conclusion: The reduction of dyslipidemia and pro-inflammatory cytokines from the of P. Aculeata extract corroborate the ethnopharmacological potential of the plant, already used by the population for hypoglycemic purposes.
Desmoplastic small round cell tumor (DSRCT) is a tumor derived from the Greek desmos referring to knot and plasis to formation an uncommon soft tissue malignant tumor, mesenchymal in origin and aggressive with a prelidiction for males and advanced at presentation. It was first described as a distinct clinical entity by Gerald WL and Rosai J (7). There are fewer than 200 reported to date. Depending on the primary site of location the Clinical manifestations vary. As most arise from the abdomen and pelvis they remain asymptomatic till they attain a huge size. Other reported sites are the skull, thorax, and paratesticular region (10,13). We report the case of a 19 yr old male who had non specific abdominal discomfort with asthenia for a period of six months and was referred to us for evaluation of left supraclavicular nodes. The prognosis of Desmoplastic small round cell tumor (DSRCT) is poor with few surviving less than two years.
The COVID-19 pandemic has shined a light on the importance of housing as a social determinant of health. To prevent millions of American renters from being evicted from their homes during the COVID-19 pandemic and potentially becoming homeless, the Centers for Disease Control and Prevention (CDC) issued a temporary eviction ban order on September 4, 2020 that has currently been extended through June 30, 2021. This paper examines the rationale for the CDC temporary eviction ban order from a public health perspective. It then uses statistical techniques to explore the relationship between political and demographic variables and temporary eviction bans enacted in individual states during the pandemic. Results show a statistically significant relationship between political party control of the state legislature and temporary state eviction bans which suggests that partisanship has largely driven the use of temporary eviction bans as a policy response to COVID-19 at the state level.
MOTION AND ITS EFFECTS ON THE CEMENT MANTLE – A BIOMECHANICAL ANALYSIS OF FEMORAL STEM DISPLACEMENT DURING IMPLANT CEMENTATION
Background: It is a common notion that motion of a femoral component during cementation should be avoided as it may weaken the cement mantle. We created an in vitro model of cemented femoral components and subjected them to varying rotational motion during the cement curing process, to measure the effect on the pullout strength of the stem. Methods: 21 sawbones femurs were separated into four groups. The first group served as control and was cemented in a standard fashion. The remainder of the stems were divided into groups and subjected to angular rotational displacement within the cement mantle during curing . Anteroposterior and lateral radiographs were obtained of each model to evaluate for cement defects. Pullout strength testing was performed. Results: Despite rotational displacement, no cement defects were noted on imaging. The control stems showed an average pullout strength of 3735.79N. The experimental groups showed a trend for lower failure loads but it was not statistically significant (P=0.063). Of the 21 stems tested, three encountered cement mantle failure and associated stem pullout and the remainder failed by peripros-thetic fracture. Conclusion: Despite conventional thinking that rotational displacement during the cementing process leading to disruption of the cement mantle integrity, this was not borne out in our study. This should give surgeons confidence that in the set-ting of unintended rotational displacement of a femoral stem, returning the stem to its original position does not significantly compromise the integrity of the cement mantle or the pullout strength of the femoral implant. Small displacement of the femoral stem with prompt correction during cement curing does not cause evident cement mantle defects or a loss of femoral stem pullout strength.
Background: Hemorrhagic cholecystitis is an infrequent complication of acute cholecystitis associated with high mortality rate. Recognition of this acute abdominal condition is often challenging. Authors present a case of acute intraluminal gallbladder bleeding with a consequential gallbladder wall perforation and hematoperitoneum requiring emergency surgery in a patient on anticoagulant therapy. Case Presentation: An 80-year old woman with atrial fibrillation on warfarin was presented to abdominal surgeon due to acute abdominal pain with hemodynamic instability and active intraluminal gallbladder bleeding on CT-scan. An emergency laparotomy with cholecystectomy was performed. Despite intensive treatment patient died on the third postoperative day. Conclusions: Hemorrhagic cholecystitis is a potentially life-threatening condition. Patients on anticoagulant therapy with clinical signs of acute cholecystitis are more prone to develop bleeding into the gallbladder. Contrast-enhanced CT of the abdomen is the diagnostic modality of choice and cholecystectomy definitive therapy in patients with hemorrhagic cholecystitis.
Multiple septic emboli and myocardial infarction due to vaso-invasive Rhizomucor pusillus infection in a hematologic patient
We present a case of a 63-year-old hematologic patient with pulmonary vaso-invasive zygomycosis with Rhizomucor pusillus after a second stem cell transplantation (SCT) for myelodysplastic syndrome, complicated by multi organ failure, myocardial ischemia and infarction. Zygomycosis is common in immunocompromised patients, especially after hematopoietic stem cell transplantation (HSCT). Mucor species have devastating vaso-invasive properties causing hematogenic dissemination. Antemortem diagnosis may be difficult due to negative cultures. Despite adequate treatment outcome tends to be poor. Cardiac zygomycosis is rare. In our patient, the clinical course and imaging results of the myocardial infarction are most consistent with coronary plaque rupture possibly provoked by severe vaso-invasive pulmonary infection and multi-organ failure.