Acute soft skull syndrome is an uncommon complication of patients with sickle cell anemia. Here, we report a case of an adult patient in Sudan with the acute soft syndrome, with our aim of providing more knowledge on this type of complication. The 20-year old patient, with a known history of sickle cell anemia, presented with a 1-day history of headache and joint pain. The complaint continued after admission, with increasing headache severity and development of rapid skull swelling, which indicated the rare sickle cell disease complication known as an acute soft head syndrome. Conservative management resulted in good response and rapid recovery of this case of acute soft skull syndrome with sickle cell anemia mainly related to skull infraction.
Systemic lupus erythematosus (SLE) is a systemic disease which affects mainly young females and can cause lifethreatening conditions. Pleural effusion can occur in SLE patients and usually tends to be mild and bilateral. This report aims to highlight the clinical presentation and medical management of massive unilateral pleural effusions in SLE patients. Here we report a 35-year-old female diagnosed with SLE for six years. She presented with shortness of breath, severe pleuritic chest pain, and fatigue. Her clinical examination showed signs of massive pleural effusion on the right side which was confirmed later by a chest x-ray and computer tomography of the chest. An echocardiography and abdominal ultrasound indicated no pericardial effusion and no ascites. A pleural fluid analysis showed exudative fluid. Sputum culture and polymerase chain reaction on blood sample for Mycobacterium tuberculosis were negative. She was also edematous and pale but not cyanotic or jaundiced. The treatment included blood transfusions, antibiotics, rituximab, azathioprine, and hydroxychloroquine. The pleural effusion responded well to rituximab, and she was discharged after two months in good condition.
Rheumatoid arthritis (RA) is a common autoimmune multi-systemic inflammatory disease affecting joints result in dysfunction and ultimately damage. Bronchiectasis (BR) is a long-term pulmonary condition that is characterized by permanent dilation of the bronchial, the association of RA and BR has been recognized since the past five decades. This is the first reported document arthropathy in a female patient with bronchiectasis in Sudan. We aim to bring attention to the uncommon presentations and challenge of diagnosing patients of BR with RA. We are presenting 48 years old female complaining of chronic cough, chest pain and multiple joints pain. examination showed Tender joints, Z-shape thumbs appearance figure and hyper flexed DIP deformity in index fingers. chest auscultation reveals bilateral Coarse crackles disappears after coughing. Chest CT scan showed features suggested the diagnosis of BR, Anti-ccp positive, rheumatoid factor IgM, all indicate a diagnosis of RA in addition, the patient has low serum Vitamin D. Patient received hydroxychloroquine, Mycophenolate mofetil, vitamin D and calcium supplements and her general condition is improved.
Jaccoud's arthropathy (JA) is a condition characterized clinically by reversible joint deformities such as ulnar deviation, swan neck, thumb subluxation and hallux valgus, along with an absence of articular erosions on a plain radiograph. This report aims to highlight the clinical presentation and medical management of JA as well as to draw attention to the disease among clinicians. We are presenting a 57-year-old male, present with a history of episodes of pain and swelling in the metacarpophalangeal, proximal and distal interphalangeal joints for 20 years associated with joint stiffness for less than half an hour during the last 15 years. Clinical examination of the hand revealed joints swelling, ulnar deviation at wrist joints and fingers, Z shape deformity of thumbs, swan neck deformity of little fingers and visible wasting of thinner muscles, although there is no tenderness or skin change and all hand deformities were correctable.
To assess and establish the relationship between neuropsychiatric systemic lupus erythematosus (NPSLE) involvement and serological biomarkers like antiribosomal-P antibodies. Patients and Methods: This is an analytical cross-sectional hospital-based study conducted on patients attending Omdurman Military Hospital from July 2019 to December 2019. A total of 90 patients were enrolled, 30 of whom had NPSLE compared with 60 SLE patients without NPSLE. SLE diagnosis was established based on the revised SLICC criteria (presence of at least 4 criteria) for SLE classification, with neuropsychiatric manifestations defined based on the ACR nomenclature. The immunological examination results have been performed by (ELISA immune-enzymatic method, immunofluorescence, and Western immunoblotting test). SPSS v 21.0 software was utilised for data analysis. Results: NPSLE patients exhibited +ve ANA in 96.7% vs 75% in non-NPSLE (P-value = 0.008), antiribosomal-P antibodies (46.7% vs 20%; P-value = 0.0001), anti-nucleosome antibodies (26.7% vs 5%; P-value = 0.005), and anti-histones antibodies (40% vs 20%; P-value = 0.04). ANA antibodies were significantly associated with neurological manifestations as ANA antibodies were common in epilepsy (n = 9; 91%) and stroke (n = 8; 27.6%) (P-value < 0.001). Conclusion: Neuropsychiatric manifestation of systemic lupus erythematosus exhibits variable clinical manifestations. Neuropsychiatric manifestations of SLE are strongly associated with the anti-ribosomal P antibody presence and can be employed as a powerful diagnostic tool.
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