Mycobacterium szulgai: A Rare Cause of Non-Tuberculous Mycobacteria Disseminated Infection.

Mycobacterium szulgai (MS) is a rare and slow-growing type of non-tuberculous mycobacteria (NTM), with a human isolation prevalence of less than 0.2% of all NTM cases. MS may cause pulmonary infection, extra-pulmonary localized disease involving the skin, lymph nodes, bone, synovial tissue or kidneys and disseminated infection, when two or more organs are affected. When disseminated infection is present, the patients usually have an underlying immunosuppressive condition. The authors report the case of a 25-year-old patient with systemic lupus erythematosus, presenting with recurrent fever, non-productive coughing, weight loss and asthenia, as well as two violaceous plaques with superficial ulceration in the gluteal region. MS was isolated from the bronchial lavage and skin biopsy cultures, confirming the rare disseminated form of MS infection. After 10 months of follow-up on isoniazid, rifampin, ethambutol and pyrazinamide, no signs of relapse were evident. To date, only 16 other cases of MS disseminated disease have been reported.

Toxic Epidermal Necrolysis-Like Lupus Erythematous Presentation Following SARS-CoV-2 Infection.

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that affects multiple organs. Infectious agents have been implicated in the pathogenesis of SLE. The emergent severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) induces a pro-inflammatory cytokine storm and has been linked to autoimmune phenomena, which can lead to the onset of autoimmune diseases. We report the case of a 70-year-old patient who developed a toxic epidermal necrolysis (TEN)-like subacute cutaneous lupus (SCL) as a severe presentation of SLE, 1 month after SARS-CoV-2 infection. After excluding other causes of SLE, treatment was initiated with a successful outcome.

[Thyroid dysfunction and amiodarone]. / Disfunção tiróidea e amiodarona.

Although most patients remain clinically euthyroid, some develop amiodarone-induced hyperthyroidism (HPEAI) or hypothyroidism (HPOAI). The authors present a retrospective analysis of ten patients with amiodarone-induced thyroid dysfunction. Six patients were female and mean amiodarone intake was 17.7 months. HPOIA was more common (six patients). From all the patients with HPEAI, two had type 2, one had type 1, and one had type 3 hyperthyroidism. Symptoms suggestive of thyroid dysfunction occurred in five patients, most of them with HPOAI. In HPEAI, the most frequent symptom was exacerbation of arrhythmia (three patients). Discontinuation of amiodarone and treatment with levothyroxine was chosen in 83.3% of the HPOAI cases, while thyonamide treatment with corticosteroids and without amiodarone was the option in 75% of the HPEAI cases. There were three deaths, all in patients with HPEAI. HPEAI is potentially fatal. The clinical picture may be vague, so the thyroid monitoring is mandatory.

Disfunção tiróidea e amiodarona / Thyroid dysfunction and amiodarone

Apesar de a maioria dos doentes tratados com amiodarona permanecer em eutiroidia, alguns desenvolvem hipertiroidismo (HPEIA) ou hipotiroidismo (HPOIA) induzidos pela amiodarona. Os autores apresentam uma análise retrospectiva dos processos de dez doentes com disfunção tiróidea induzida pela amiodarona. Verificou-se que seis doentes eram mulheres e que o tempo médio de toma da amiodarona foi de 17,7 meses. O HPOIA foi o mais frequente (seis doentes). Dos doentes com HPEIA, dois tinham HPEIA tipo 2, um tipo 1 e um tipo 3. Sintomas sugestivos de disfunção tiróidea ocorreram em cinco doentes, a maioria com HPOIA. No HPEIA, a clínica mais comum foi exacerbação da arritmia de base (três doentes). A interrupção da amiodarona e administração de levotiroxina foi a terapêutica escolhida em 83,3% dos casos de HPOIA, enquanto a tionamida associada a corticoide com suspensão da amiodarona foi opção em 75% dos casos de HPEIA. Registraram-se três óbitos, todos com HPEIA. O HPEIA constituiu uma complicação potencialmente fatal. A clínica pode ser vaga, pelo que a monitorização da função tiróidea é obrigatória.

Although most patients remain clinically euthyroid, some develop amiodarone-induced hyperthyroidism (HPEAI) or hypothyroidism (HPOAI). The authors present a retrospective analysis of ten patients with amiodarone-induced thyroid dysfunction. Six patients were female and mean amiodarone intake was 17.7 months. HPOIA was more common (six patients). From all the patients with HPEAI, two had type 2, one had type 1, and one had type 3 hyperthyroidism. Symptoms suggestive of thyroid dysfunction occurred in five patients, most of them with HPOAI. In HPEAI, the most frequent symptom was exacerbation of arrhythmia (three patients). Discontinuation of amiodarone and treatment with levothyroxine was chosen in 83.3% of the HPOAI cases, while thyonamide treatment with corticosteroids and without amiodarone was the option in 75% of the HPEAI cases. There were three deaths, all in patients with HPEAI. HPEAI is potentially fatal. The clinical picture may be vague, so the thyroid monitoring is mandatory.

Predictors of in-hospital mortality in elderly patients with bacteraemia admitted to an Internal Medicine ward.

BACKGROUND: Infectious diseases are a common cause of increased morbidity and mortality in elderly patients. Bacteraemia in the elderly is a difficult diagnosis and a therapeutic challenge due to age-related vicissitudes and to their comorbidities. The main purpose of the study was to assess independent risk factors for in-hospital mortality among the elderly with bacteraemia admitted to an Internal Medicine Ward. METHODS: Overall, a cohort of 135 patients, 65 years of age and older, with bacteraemia were retrospectively studied. Data related to demographic information, comorbidities, clinical parameters on admission, source and type of infection, microorganism isolated in the blood culture, laboratory data and empirical antibiotic treatment was recorded from each patient. Multivariate logistic regression was performed to identify independent predictors of all-cause in-hospital mortality. RESULTS: Of these 135 patients, 45.9% were women. The most common infections in this group of patients were urinary tract infections (46.7%). The main microorganisms isolated in the blood cultures were Escherichia coli (14.9%), Methicillin-resistant Staphylococcus aureus (MRSA) (12.0%), non-MRSA (11.4%), Klebsiella pneumoniae (9.1%) and Enterococcus faecalis (8.0%). The in-hospital mortality was 22.2%. Independent prognostic factors associated with in-hospital mortality were age ≥ 85 years, chronic renal disease, bacteraemia of unknown focus and cognitive impairment at admission (OR, 2.812 [95% CI, 1.039-7.611; p = 0.042]; OR, 6.179 [95% CI, 1.840-20.748; p = 0.003]; OR, 8.673 [95% CI, 1.557-48.311; p = 0.014] and OR, 3.621 [95% CI, 1.226-10.695; p = 0.020], respectively). By multivariate analysis appropriate antibiotic therapy was not associated with lower odds of mortality. CONCLUSION: Bacteraemia in the elderly has a high mortality rate. There are no set of signs or clinical features that can predict bacteraemia in the elderly. However, older age (≥ 85 years), chronic renal disease, bacteraemia of unknown focus and severe cognitive impairment adversely affects the outcome of elderly patients with bacteraemia admitted to an Internal Medicine ward.

The intriguing co-existence of a chronic periaortitis, a pericarditis and a pancreatitis: case report.

Chronic periaortitis (CP) refers to a spectrum of diseases whose common denominator is a fibro-inflammatory tissue developing in the periaortic space and frequently encasing surrounding structures like the kidney and ureters. There is no unified concept regarding the primary aetiology of CP, but recent studies have demonstrated that CP may present features of auto-immune diseases. CP involves three main entities, namely idiopathic retroperitoneal fibrosis (IRF), inflammatory aneurysms of the abdominal aorta (IAAAs) and perianeurysmal retroperitoneal fibrosis (PRF). These entities are usually diagnosed using computed tomography or magnetic resonance imaging, which typically show a retroperitoneal mass surrounding the aorta and that extends laterally without displacing it. Positron emission tomography is useful for the full assessment of the extent of the disease and its metabolic activity. The inflammatory and chronic relapsing nature of these diseases compels the use of medical therapy, which is based on high-dose steroids with a tapering scheme combined with immunosuppressive agents in refractory or relapsing disease. The authors report the clinical and radiological characteristics of a nonaneurysmatic form of chronic periaortitis in a woman presented with pericarditis, pericardial effusion and a pancreatitis. They also describe the investigation and management of this unusual condition.

An unusual presentation of osteogenesis imperfecta type I.

Osteogenesis imperfecta (OI) is a rare inherited disorder with a broad spectrum of clinical and genetic variability. The genetic diversity involves, in the majority of the cases, mutations in one of the genes that encodes the type 1 collagen protein (COL1 A1 and COL1 A2), but it is not a requirement for the diagnosis. The most benign form is OI type I. The authors present a case report of a 25-year-old woman who had severe low back pain associated with incapacity to walk and breast-feed post-partum. Symptoms developed 2 weeks after delivery. The radiological examination revealed severe osteoporosis with no abnormalities in the laboratory findings. The clinical signs and a positive personal and family history of multiple fractures in childhood suggested OI type I, although other diagnosis, such as pregnancy-associated osteoporosis, was also considered. The atypical presentation of this rare disorder in adulthood calls attention to the need for early diagnosis for prompt treatment. Treatment of OI is never curative, but it improves the quality of the patient's life.

Ehlers-Danlos syndrome type IV in association with a (c.970G>A) mutation in the COL3A1 gene.

The Ehlers-Danlos syndrome type IV (EDS-IV) is a hereditary, autosomal dominant disease that causes a defect in the procollagen III synthesis, which results in a structural modification in this protein. An awareness of the disease is of vital importance for the optimal outcome, since the affected individuals have a high risk of vascular, intestinal and uterine rupture. It's a disease with great clinical variability and the diagnosis is confirmed by detection of a mutation in the gene encoding collagen type III. The authors present a case report of a patient who appeared at the emergency ward with acute abdomen and hypovolemic shock after spontaneous aortic rupture. The diagnosis was confirmed after genetic study that identified a mutation in the (c.970G>A) in the COL3A1 gene, only reported once in the literature in a family with internal carotid dissections in some of its members. It's the first time that this mutation is reported in association with the EDS-IV. The authors also make a brief review of the clinical, genetic and molecular characteristics of this syndrome.