COVID-19 in patients with rheumatological diseases in the Eastern Province of Saudi Arabia.

The severity of the 2019 coronavirus disease (COVID-19) and its effects remain unpredictable. Certain factors, such as obesity, hypertension, and type 2 diabetes mellitus, may increase the severity of the disease. Rheumatology experts suggest that patients with active autoimmune conditions and controlled autoimmune diseases on immunosuppressive therapy may be at higher risk of developing severe COVID-19. In this retrospective observational study, we aimed to examine the patterns of COVID-19 in patients with underlying rheumatological diseases and their association with disease severity and hospital outcomes. A total of 34 patients with underlying rheumatological diseases who tested positive for severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) by polymerase chain reaction (PCR) were included between March 2020 and April 2021 at King Fahd Hospital of the University. The study population consisted of 76.47% female and 23.53% male patients, with a mean age ranging from 20 to 40 years. Female gender (p=0.0001) and younger age (p=0.004) were associated with milder disease. The most frequent rheumatological disease was systemic lupus erythematosus (SLE) (38.24%), which was associated with a milder infection (p=0.045). Patients treated with mycophenolate mofetil (MMF) had a milder disease course (p=0.0037). Hypertension was significantly associated with severe COVID-19 disease (p=0.037). There was no significant relationship between SLE and the need for ICU admission. Patients on hydroxychloroquine and MMF tended to develop milder disease, and there was no association between the severity of the infection and the treatment with steroids.

Clinical outcome of pre-operative blood transfusion for sickle cell disease patients in post-operative complications.

BACKGROUND: Preoperative blood transfusion for patients with sickle cell disease is a debatable topic and it can be lifesaving. Sickle cell disease patients are at high risk for vaso-occlusive crisis due to the large concentration of sickle hemoglobin (HgbS) in their blood. Despite the current extensive research into this disease, there is still no consensus over whether blood transfusion is a preferable preoperative modality among patients undergoing elective surgical procedures. METHOD: A retrospective observational study, which enrolled 204 patients with Sickle cell disease who underwent surgery at King Fahad Hospital of the University (KFHU) over the last five years. The primary objective was to determine whether there is evidence that preoperative blood transfusion for SCD patients undergoing surgical procedures will reduce postoperative complications related to SCD. RESULTS: A total of 204 patients were included, of which 30% had preoperative blood transfusion. Majority of patient 44% had undergone cholecystectomy. On multivariate logistic regression analysis, patients who did not undergo blood transfusion had significantly higher risk to develop post-operative SCD complications (OR = 3.07, P value = 0.002). In addition, they had significantly prolonged hospitalization (OR = 2.22, P value = 0.08). In contrast, patients who received blood transfusion had lower risk for developing post-operative SCD-related complications (OR = 1.87, P value = 0.29), and decrease in the duration of hospitalization by (OR = 0.49, P value = 0.045). CONCLUSION: Our study showed that patients who had not undergone preoperative blood transfusion had higher risk to develop postoperative complications and prolonged hospital stay compared to those who underwent blood transfusion.

Baseline MRI findings as predictors of hypopituitarism in patients with non-functioning pituitary adenomas.

Hypopituitarism tends to occur in large pituitary adenomas. However, similar tumors could present with strikingly different hormonal deficiencies. In this study, we looked at MRI characteristics in non-functioning pituitary adenomas (NFPA), which could predict secondary adrenal insufficiency (SAI) and central hypothyroidism (CHT). We reviewed the files of patients with NFPA attending our clinic. Tumor size, invasiveness, MR-signal intensity, and gadolinium enhancement in preoperative MRI were recorded along with documented presurgical hypopituitarism profile. Logistic regression was used to predict SAI, CHT, or both (SAI/CHT) based on MRI and demographic parameters. Receiver operating characteristic curves were used to determine their diagnostic utility. One hundred twenty-one patients were included in the study. Older age (P = 0.021), male sex (P = 0.043), stalk deviation (P < 0.0001), contrast enhancement (P = 0.029), and optic chiasma compression (P = 0.012) were associated with SAI/CHT. Adenoma vertical height, largest diameter, and estimated volume were also strongly associated with SAI/CHT (P < 0.0001). These associations remained significant in a multivariate analysis. No tumor smaller than 12 mm in vertical height, 17 mm in largest diameter, or 0.9 cm3 in volume was associated with SAI/CHT. At cut-off ≥18 mm for vertical height, ≥23 mm for largest diameter, and ≥3.2 cm3 the sensitivity was around 90-92% for detecting SAI/CHT. Only vertical height was significantly associated with any one or more pituitary hormonal deficit (P = 0.001). In conclusion, adenoma size, independent of the measurement used, remains the best predictor of SAI/CHT in NFPA. Dynamic testing to rule out SAI is probably indicated in adenomas larger than 18 mm vertical height, 23 mm largest diameter and 3.2 cm3 adenoma volume.

Liver Metastasis in a Young Female Secondary to Breast Cancer: A Case Report.

Breast cancer is common among females worldwide and is most commonly reported in women aged 30-40 years and less commonly in those aged <30 years. Presentation with liver metastasis is rare in breast cancer at all ages. Lactic acidosis in association with metastatic breast cancer is also rare. Here, the authors report a case of a 26-year-old female who presented with cholestatic jaundice, coagulopathy and ascites. Radiological examination showed evidence of infiltrating liver lesion. Computed tomography-guided liver biopsy confirmed the diagnosis of adenocarcinoma of breast origin based on the strong estrogen receptor positivity. Chemotherapy could not be initiated because of the patient's critical condition. Unfortunately, the condition deteriorated rapidly, and the patient died secondary to liver failure manifested with disseminated intravascular coagulation and lactic acidosis. This is a rare case of breast cancer in terms of age group (<30 years), site of metastasis at presentation and complication of metastatic breast cancer (type B lactic acidosis), and thus highlights the distinct features of such breast cancers.

Glucocorticoid resistance syndrome caused by a novel NR3C1 point mutation.

Glucocorticoid resistance syndrome (GRS) is a rare genetic disorder caused by inactivating mutations of the NR3C1 gene which encodes the glucocorticoid receptor. The phenotypic spectrum is broad but typically include symptoms of adrenal insufficiency, mineralocorticoid excess and hyperandrogenism. We report a new case associated with a novel NR3C1 mutation. A 55-year-old woman with lifelong history of low body weight, hyperandrogenism and anxiety was seen at the endocrine clinic after left adrenalectomy and salpingoophorectomy for lesions suspicious of ovarian cancer and adrenal metastasis. The tumors turned out to be a 3.5 cm benign ovarian serous adenofibroma and a 3.5 cm multinodular adrenal mass. She complained of worsened fatigue and inability to recover weight lost with surgery. Pre-operative serum and urinary cortisol were elevated, but she had no stigma of Cushing's syndrome. Plasma ACTH was elevated and a 1-mcg cosyntropin stimulation test was normal. Her fatigue persisted over ensuing years and ACTH-dependent hypercortisolemia remained stable. Low dose oral dexamethasone failed to suppress endogenous cortisol. A pituitary MRI was normal but revealed incidental brain aneurysms. Bone densitometry showed profound osteoporosis. On the bases of this contradictory clinical picture, glucocorticoid resistance syndrome (GRS) was suspected. Using next generation sequencing technology, a novel heterozygous pathogenic variant in the NR3C1 gene was detected. We speculate that vascular malformations and profound osteoporosis, findings associated to cortisol excess, reflect in our patient a variable tissue sensitivity to glucocorticoids. In conclusion, in patients with clinically unexpected ACTH-dependent hypercortisolemia, primary glucocorticoid resistance (GRS) should be considered.

Tuberculosis-associated Immune Thrombocytopenia: A Case Report.

Various hematological manifestations are known to occur with tuberculosis (TB), but its association with immune thrombocytopenia is uncommon and not well recognized. Here, the case of a 39-year-old male who presented with a history of epistaxis and hematuria is described. The patient was found to have diffuse lymphadenopathy both clinically and radiologically. He was diagnosed with immune thrombocytopenia; however, there was a delay in the diagnosis of TB because of the patient's refusal of lymph node biopsy and late recognition of the association between TB and immune thrombocytopenia. Treatment with steroids without antituberculosis medications may have led to reactivation and dissemination of tuberculous infection in this patient. Later, the patient was readmitted with a suspected community-acquired pneumonia and the sputum smear was positive for acid-fast bacilli. Unfortunately, the patient died after he developed sepsis and multiorgan failure. The purpose of this case report is to highlight this rare combination and create awareness among clinicians to consider TB as an underlying etiology of immune thrombocytopenia, especially if there are other associated physical findings such as the presence of lymphadenopathy.