Severe primary hypothyroidism and ovarian hyperstimulation syndrome in a spontaneous pregnancy: a case report.

Background: Ovarian hyperstimulation syndrome (OHSS) is a rare ovulation induction therapy side effect. Nevertheless, it can occur in spontaneous ovulation cycles linked to multiple gestation, molar pregnancy, polycystic ovarian syndrome, and hypothyroidism. The pathogenesis of OHSS remains poorly understood. However, in recent studies, it has been observed that increased concentrations of thyroid-stimulating hormone (TSH) can potentially have stimulatory effects on the ovaries due to the homologous structure shared between TSH and gonadotropins. It is recommended to delay pregnancies until euthyroidism is achieved with replacement therapy to reduce potentially fatal problems. Case Description: We describe the case of a 22-year-old female patient who sought medical attention due to a 4-week history of abdominal discomfort and amenorrhea. Upon evaluation, it was determined that she was in the 9th week of pregnancy and experiencing OHSS due to severe primary hypothyroidism. The diagnosis was confirmed through laboratory and imaging data, enabling timely care and preventing complications arising from unwarranted surgical intervention. Administration of levothyroxine led to total regression of the ovarian cysts. Even so, the patient decided to terminate her pregnancy. Conclusions: This case illustrates the occurrence of OHSS in a woman with untreated hypothyroidism. Notably, this syndrome is relatively uncommon, and the patient's ability to conceive while having unviable thyroid hormone levels further adds to this case's exceptional nature.

Acute paraplegia in a patient with eosinophilic granulomatosis and polyangiitis with 20 years of evolution: case report.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare and necrotizing vasculitis of small and medium vessels with a heterogeneous presentation, multiorgan involvement, characterized by the presence of chronic rhinosinusitis, asthma, and peripheral eosinophilia. Nervous system involvement is frequent, especially in the peripheral nervous system (PNS), in the form of mononeuritis multiplex. However, subarachnoid and cerebral hemorrhage, cerebral infarction, cranial nerve palsies, and cortical blindness have also been reported in the literature. Initial therapy involves systemic GC, which are not free of complications. We present the case of a patient with EGPA of more than 20 years duration who suddenly developed bilateral paresthesia of the lower limbs and urinary retention. The neurological exam revealed paraplegia with a sensorial level in D4; the MRI showed spinal cord compression in the D2-D7 level, and the patient was emergently submitted to surgical decompression. The histopathology of the surgical specimen demonstrated the presence of epidural adipose tissue with multiple vasculitic lesions. The effects of systemic corticosteroid therapy may contribute to abnormal fat deposition in various body segments, including the neuroaxis, leading to the development of epidural lipomatosis. However, the intra-lesional vasculitic character is a unique manifestation of myelopathy that has shown us to have a more aggressive attitude.

Spontaneous Primary Pneumothorax as A Complication of Neurofibromatosis Type 1.

Neurofibromatosis type 1 (NF1) is a genetic disorder that affects the skin and the neurological, ocular and skeletal systems. Many are unaware of the extent of pulmonary involvement, including lung cysts and emphysematous bullae, which enhances the risk of secondary spontaneous pneumothorax (SSP). We report the case of an 18-year-old male with NF1 who presented with acute dyspnoea and chest pain due to a right-sided pneumothorax caused by the rupture of lung apical bullae. The patient received supplemental oxygen and a chest tube of 18F was inserted, with a complete resolution of the pneumothorax. He was discharged on the third day of hospital stay. This case highlights the importance of considering SSP as a possible clinical manifestation and complication of NF1. Early recognition and appropriate management of this condition can prevent serious complications and improve patient outcomes. LEARNING POINTS: NF1 is a genetic disease that results in cutaneous conditions, including neurofibroma, axillary, inguinal lentigines and café au lait spots. In some cases (5-20%) it can also affect the lungs, causing neurofibroma, infiltrative and cystic lesions, emphysema or bullae, leading to chronic respiratory failure.SSP is a clinical presentation of NF1 caused by the rupture of lung cysts or bullae, with an unclear relationship to smoking.Early diagnosis of pulmonary manifestations in patients with NF1 is crucial as surgical removal of lung cysts and bullae seems to prevent recurrence of SSP.

Biological therapy in systemic lupus erythematosus, antiphospholipid syndrome, and Sjögren's syndrome: evidence- and practice-based guidance.

Systemic lupus erythematosus (SLE), antiphospholipid syndrome (APS), and Sjögren's syndrome (SS) are heterogeneous autoimmune diseases. Severe manifestations and refractory/intolerance to conventional immunosuppressants demand other options, namely biological drugs, and small molecules. We aimed to define evidence and practice-based guidance for the off-label use of biologics in SLE, APS, and SS. Recommendations were made by an independent expert panel, following a comprehensive literature review and two consensus rounds. The panel included 17 internal medicine experts with recognized practice in autoimmune disease management. The literature review was systematic from 2014 until 2019 and later updated by cross-reference checking and experts' input until 2021. Preliminary recommendations were drafted by working groups for each disease. A revision meeting with all experts anticipated the consensus meeting held in June 2021. All experts voted (agree, disagree, neither agree nor disagree) during two rounds, and recommendations with at least 75% agreement were approved. A total of 32 final recommendations (20 for SLE treatment, 5 for APS, and 7 for SS) were approved by the experts. These recommendations consider organ involvement, manifestations, severity, and response to previous treatments. In these three autoimmune diseases, most recommendations refer to rituximab, which aligns with the higher number of studies and clinical experience with this biological agent. Belimumab sequential treatment after rituximab may also be used in severe cases of SLE and SS. Second-line therapy with baricitinib, bortezomib, eculizumab, secukinumab, or tocilizumab can be considered in SLE-specific manifestations. These evidence and practice-based recommendations may support treatment decision and, ultimately, improve the outcome of patients living with SLE, APS, or SS.

Biologic therapy in large and small vessels vasculitis, and Behçet's disease: Evidence- and practice-based guidance.

OBJECTIVE: Vasculitis are a very heterogenous group of systemic autoimmune diseases, affecting large vessels (LVV), small vessels or presenting as a multisystemic variable vessel vasculitis. We aimed to define evidence and practice-based recommendations for the use of biologics in large and small vessels vasculitis, and Behçet's disease (BD). METHODS: Recommendations were made by an independent expert panel, following a comprehensive literature review and two consensus rounds. The panel included 17 internal medicine experts with recognized practice on autoimmune diseases management. The literature review was systematic from 2014 until 2019 and later updated by cross-reference checking and experts' input until 2022. Preliminary recommendations were drafted by working groups for each disease and voted in two rounds, in June and September 2021. Recommendations with at least 75% agreement were approved. RESULTS: A total of 32 final recommendations (10 for LVV treatment, 7 for small vessels vasculitis and 15 for BD) were approved by the experts and several biologic drugs were considered with different supporting evidence. Among LVV treatment options, tocilizumab presents the higher level of supporting evidence. Rituximab is recommended for treatment of severe/refractory cryoglobulinemic vasculitis. Infliximab and adalimumab are most recommended in treatment of severe/refractory BD manifestations. Other biologic drugs can be considered is specific presentations. CONCLUSION: These evidence and practice-based recommendations are a contribute to treatment decision and may, ultimately, improve the outcome of patients living with these conditions.

Acute meningitis complicated with ventriculitis caused by Streptococcus dysgalactiae subsp. dysgalactiae.

Streptococcus dysgalactiae subspecies dysgalactiae (SDSD), also known as Lancefield group C streptococcus, is a pathogen found in animals. It is known to cause pyogenic infections in animals and is one of the most common pathogens that can cause mastitis in cattle. Very few reports of SDSD causing human diseases to have been reported in the literature, but we report a case of community-acquired meningitis and pyogenic ventriculitis caused by SDSD. This report is the first case of SDSD causing a central nervous system (CNS) infection in humans and aims to raise awareness about the role of SDSD in CNS infections. It also seeks to promote the recognition of this bacteria as a potential cause of invasive diseases.

Fulminans Purpura due to Morganella morganii.

Purpura fulminans (PF) is a dermatological manifestation of a life-threatening condition characterized by disseminated intravascular coagulation and endovascular thrombosis. The idiopathic/infectious form is the most common and usually associated with infection by Neisseria meningitidis or Streptococcus pneumoniae. We describe a case of Morganella morganii-induced bacteriaemia complicated with PF in an individual who had undergone a recent urinary tract infection intervention. The patient presented with purpuric skin lesions, fever and hypotension but had no alterations in coagulation parameters or disseminated intravascular coagulation. Aggressive early resuscitation, intravenous antibiotics and wound care were essential to a favourable response. LEARNING POINTS: Purpura fulminans is a dermatological manifestation of an underlying life-threatening condition, and is characterized by disseminated intravascular coagulation and skin necrosis.It is a morbid and potentially fatal condition that can be a cutaneous manifestation of Morganella morganii bacteraemia.Early identification and accurate diagnosis of the underlying cause can help minimize morbidity and mortality; management should be tailored to the individual, with the use of intravenous antibiotics, necrotic skin excision and aggressive early resuscitation.

Síndrome febril indeterminado y paniculitis – un caso raro de linfoma T subcutáneo paniculitis-like / Undetermined febrile syndrome and panniculitis - a rare case of subcutaneous panniculitis-like T-cell lymphoma

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is an extremely rare form of skin lymphoma that primarily involves subcutaneous adipose tissue. SPTCL diagnosis is demanding because of its nonspecific systemic features, such as fever or weight loss, that usually mimic other more common conditions. Further complicating diagnosis, lesion biopsies are often inconclusive. For this matter, patients are frequently seen by different clinicians and may be submitted to various cutaneous biopsies before a definitive diagnosis is reached. We present the case of a 64-year-old man with a two-month history of fever and subcutaneous nodular lesions scattered by the lower limbs and torso, whose final diagnosis of SPTCL illustrates the main features of the disease as well as the challenge of its identification. (AU)

Incidence of Antibiotic Treatment Failure in Patients with Nursing Home-Acquired Pneumonia and Community Acquired Pneumonia.

PURPOSE: Nursing home-acquired pneumonia (NHAP) patients are at higher risk of multi-drug resistant infection (MDR) than those with community-acquired pneumonia (CAP). Recent evidence suggests a single risk factor for MDR does not accurately predict the need for broad-spectrum antibiotics. The goal of this study was to compare the rate antibiotic failure between NHAP and CAP patients. METHODS: Demographic characteristics, co-morbidities, clinical and laboratory variables, antibiotic therapy, and mortality data were collected retrospectively for all patients with pneumonia admitted to an Internal Medicine Service between April 2017 and April 2018. RESULTS: In total, 313 of 556 patients had CAP and 243 had NHAP. NHAP patients were older, and were more likely to be dependent, to have recent antibiotic use, and to experience treatment failure (odds ratio (OR) 1.583; 95% CI 1.102-2.276; p = 0.013). In multivariate analysis, patient's origin did not predict treatment failure (OR 1.083; 95% CI 0.726-1.616; p = 0.696). DISCUSSION: Higher rates of antibiotic failure and mortality in NHAP patients were explained by the presence of other risk factors such as comorbidities, more severe presentation, and age. Admission from a nursing home is not a sufficient condition to start broader-spectrum antibiotics.

Adult pulmonary Langerhans cell histiocytosis revealed by central diabetes insipidus: A case report and literature review.

Langerhans cell histiocytosis (LCH) is a rare systemic and heterogeneous disease secondary to proliferation and diffuse infiltration of immature CD1a-positive dendritic cells, also known as Langerhans cells. LCH affects predominantly paediatric patients and is rarely diagnosed in adulthood. Despite its worldwide prevalence, most reported cases are found in the Japanese population. There is no consensus regarding treatment strategy due to the low incidence of this disease and the diversity of symptoms that appear. An integrative literature review was conducted based on the PubMed database using MeSH terms 'Langerhans', 'histiocytosis' and 'adult'. The present report describes a case of a successfully treated LCH-induced central diabetes insipidus (uncommon presentation in adult patients) as well as an updated review of current evidence published on this matter.

Hyperglycaemic Hemichorea and Hyperglycaemic Hyperosmolar Syndrome in a Patient with HbA1c levels of 19.9%: A Rare and Potentially Reversible Case.

Hemichorea is a rare clinical manifestation of type 2 diabetes mellitus. The patient presents with non-ketotic hyperglycaemia, hemichorea (characterized by rapid and involuntary movements of a specific part of the body) and the CT imaging reveals the presence of alterations involving the ganglia of the base[1-3]. LEARNING POINTS: Hemichorea is a rare clinical manifestation of type 2 diabetes mellitus associated with an uncontrolled disease.Hyperglycaemic hemichorea has a good prognosis.All diabetics need to maintain good metabolic control and control cardiovascular risk factors.

Keeping track of migratory pulmonary lesions.

Tricuspid valve endocarditis (TVE) is rarely considered in the differential diagnosis of a febrile patient who does not use intravenous drugs.We describe the case of a 62-year-old male patient with a 3-month history of remittent fever and 13% weight loss. The patient denied intravenous drugs use or recent invasive procedures. His medical history included type 2 diabetes, alcohol abuse and smoking. Clinical evaluation revealed systemic inflammatory syndrome with unremarkable physical examination. Ancillary tests showed leucocytosis, thrombocytopenia and elevated C reactive protein. Empiric intravenous ceftriaxone was started, but after an initial improvement, fever relapsed 2 days after stopping antibiotherapy. A CT scan showed multiple disseminated lesions, suggesting lung metastatic tumour. Further studies excluded malignancy and revealed TVE caused by Streptococcus bovis with pulmonary embolism. The aim of our study is to stress the importance of evoking TVE in the differential diagnosis of fever with lung manifestations, and to highlight the possible association between S bovis, colorectal cancer and liver disease.

Emergent biomarkers of residual cardiovascular risk in patients with low HDL-c and/or high triglycerides and average LDL-c concentrations: focus on HDL subpopulations, Oxidized LDL, adiponectin, and uric acid.

This study intended to determine the impact of HDL-c and/or TGs levels on patients with average LDL-c concentration, focusing on lipidic, oxidative, inflammatory, and angiogenic profiles. Patients with cardiovascular risk factors (n = 169) were divided into 4 subgroups, combining normal and low HDL-c with normal and high TGs patients. The following data was analyzed: BP, BMI, waist circumference and serum glucose, Total-c, TGs, LDL-c, oxidized-LDL, total HDL-c and HDL subpopulations, paraoxonase-1 (PON1) activity, hsCRP, uric acid, TNF- α , adiponectin, VEGF, and iCAM1. The two populations with increased TGs levels, regardless of the normal or low HDL-c, presented obesity and higher waist circumference, Total-c, LDL-c, Ox-LDL, and uric acid. Adiponectin concentration was significantly lower and VEGF was higher in the population with cumulative low values of HDL-c and high values of TGs, while HDL quality was reduced in the populations with impaired values of HDL-c and/or TGs, viewed by reduced large and increased small HDL subfractions. In conclusion, in a population with cardiovascular risk factors, low HDL-c and/or high TGs concentrations seem to be associated with a poor cardiometabolic profile, despite average LDL-c levels. This condition, often called residual risk, is better evidenced by using both traditional and nontraditional CV biomarkers, including large and small HDL subfractions, Ox-LDL, adiponectin, VEGF, and uric acid.

Implication of low HDL-c levels in patients with average LDL-c levels: a focus on oxidized LDL, large HDL subpopulation, and adiponectin.

To evaluate the impact of low levels of high density lipoprotein cholesterol (HDL-c) on patients with LDL-c average levels, focusing on oxidative, lipidic, and inflammatory profiles. Patients with cardiovascular risk factors (n = 169) and control subjects (n = 73) were divided into 2 subgroups, one of normal HDL-c and the other of low HDL-c levels. The following data was analyzed: BP, BMI, waist circumference and serum glucose Total-c, TGs, LDL-c, oxidized LDL, total HDL-c and subpopulations (small, intermediate, and large), paraoxonase-1 (PON1) activity, hsCRP, uric acid, TNF- α , adiponectin, VEGF, and iCAM1. In the control subgroup with low HDL-c levels, significantly higher values of BP and TGs and lower values of PON1 activity and adiponectin were found, versus control normal HDL-c subgroup. However, differences in patients' subgroups were clearly more pronounced. Indeed, low HDL-c subgroup presented increased HbA1c, TGs, non-HDL-c, Ox-LDL, hsCRP, VEGF, and small HDL-c and reduced adiponectin and large HDL. In addition, Ox-LDL, large-HDL-c, and adiponectin presented interesting correlations with classical and nonclassical markers, mainly in the normal HDL-c patients' subgroup. In conclusion, despite LDL-c average levels, low HDL-c concentrations seem to be associated with a poor cardiometabolic profile in a population with cardiovascular risk factors, which is better evidenced by traditional and nontraditional CV biomarkers, including Ox-LDL, large HDL-c, and adiponectin.

Diabetes abrogates sex differences and aggravates cardiometabolic risk in postmenopausal women.

BACKGROUND: The aim of this study is to evaluate the effect of gender and menopause in cardiometabolic risk in a type 2 diabetes mellitus (T2DM) population, based on classical and non-traditional markers. METHODS: Seventy four volunteers and 110 T2DM patients were enrolled in the study. Anthropometric data, blood pressure, body mass index (BMI), waist circumference (WC) and the following serum markers were analyzed: glucose, Total-c, TGs, LDL-c, Oxidized-LDL, total HDL-c and large and small HDL-c subpopulations, paraoxonase 1 activity, hsCRP, uric acid, TNF-α, adiponectin and VEGF. RESULTS: Non-diabetic women, compared to men, presented lower glycemia, WC, small HDL-c, uric acid, TNF-α and increased large HDL-c. Diabetes abrogates the protective effect of female gender, since diabetic women showed increased BMI, WC, small HDL-c, VEGF, uric acid, TNF-α and hsCRP, as well as reduced adiponectin, when compared with non-diabetic. In diabetic females, but not in males, WC is directly and significantly associated with TNF-α, VEGF, hsCRP and uric acid; TNF-α is directly associated with VEGF and hsCRP, and inversely with adiponectin. Postmenopausal females presented a worsen cardiometabolic profile, viewed by the increased WC, small HDL-c, VEGF, uric acid, TNF-α and hsCRP. In this population, WC is directly and significantly associated with TNF-α, VEGF, hsCRP; TNF-α is directly associated with VEGF; and uric acid is inversely associated with large HDL-c and hsCRP with adiponectin, also inversely. CONCLUSIONS: Diabetes abrogates the protective effect of gender on non-diabetic women, and postmenopausal diabetic females presented worsen cardiometabolic risk, including a more atherogenic lipid sketch and a pro-inflammatory and pro-angiogenic profile. The classical cardiovascular risk factors (CVRFs) fail to completely explain these differences, which are better clarified using "non-traditional" factors, such as HDL-c subpopulations, rather than total HDL-c content, and markers of inflammation and angiogenesis, namely TNF-α, hsCRP, uric acid and VEGF. Multi-therapeutic intervention, directed to obesity, atherogenic lipid particles and inflammatory mediators is advisory in order to efficiently prevent the serious diabetic cardiovascular complications.

New markers of early cardiovascular risk in multiple sclerosis patients: oxidized-LDL correlates with clinical staging.

OBJECTIVES: This study aimed to characterize a population of multiple sclerosis (MS) patients in terms of traditional and new cardiovascular risk factors and assess their putative correlation with clinical disease activity (evaluated by the Expanded Disability Status Scale [EDSS]). METHODS: Thirty relapsing MS patients and 66 subjects, matched by age and sex, were enrolled in this cross-sectional study. For each subject, anthropometric data were collected and classical biochemical (including lipid profile, glucose and C reactive protein [CRP] levels) and novel markers (paraoxonase 1 [PON1] enzyme activity and contents of high-density lipoprotein [HDL] cholesterol, oxidized low-density lipoprotein [Ox-LDL], tumor necrosis factor [TNF]-alfa, vascular endothelial growth factor [VEGF] and adiponectin) were studied. RESULTS: In patients group, 23 women and 7 men were included, aged 35.00 (28.25-40.25) years and scoring a median of 2.00 (1.50-3.13) in EDSS. Comparing with controls, the most relevant differences encountered were: increased serum triglycerides (P< 0.001), Ox-LDL (P< 0.001) as well as Ox-LDL/LDL ratio and reduced small HDL (P=0.040), accompanied by a trend to increased VEGF concentration. LDL content, especially Ox-LDL, showed positive and significant correlation with EDSS (r=0.458; P=0.011) and VEGF (r=0.453; P=0.014). CONCLUSIONS: MS patients presented a profile of early CV risk, being Ox-LDL contents a putative good marker and having correlation with the clinical activity of the disease.