Τετάρτη 24 Φεβρουαρίου 2021

Adult-onset Still's disease evolving with multiple organ failure and death: A case report and review of the literature

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World J Clin Cases. 2021 Feb 6;9(4):886-897. doi: 10.12998/wjcc.v9.i4.886.

ABSTRACT

BACKGROUND: Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disease, which is characterized by daily fever and arthritis, with an evanescent rash and neutrophilic leukocytosis. To date, there has been no definite laboratory or imaging test available for diagnosing AOSD; the diagnosis is one of exclusion, which can be very challenging. In particular, AOSD patients may experience different complications affecting their clinical picture, management, and prognosis. The treatment of AOSD remains largely empirical and involves therapeutic agents.

CASE SUMMARY: We report the case of a 36-year-old woman who presented with fever, red rash, arthralgia, and sore throat. Her serum ferritin level and white blood cell count were markedly elevated, and the first diagnosis 22 years prior was "juvenile rheumatoid arthritis of systemic type". The patient was treated with prednisone, sulfasalazine, methotrexate, and leflunomide. After remission of her symptoms, the patient stopped taking the medications, and the disease recurred. Ultimately, the patient was diagnosed with adult-onset Still's disease. Relapse occurred several times due to self-medication withdrawal, and an interleukin-6 antagonist (tocilizumab/Actemra) was administered to control the disease. Recently, she was hospitalized because an incision did not heal, and the patient suddenly developed high fever and diarrhea during hospitalization. The patient's disease progressed violently and quickly developed into macrophage activation syndrome, disseminated intravascular coagulation, shock, and multiple organ failure. The patient had sudden cardiac arrest, and she died despite emergency rescue efforts.

CONCLUSION: AOSD patients need regular follow-up in the long-term treatment process, and must press formulary standard medication, and do not voluntarily withdr aw or reduce the dose. Otherwise it may cause disease back-and-forth or serious life-threatening complications. Meanwhile, strict management of trauma, infections, tumors, and other diseases may contribute to improved outcomes in patients with complications.

PMID:33585636 | PMC:PMC7852636 | DOI:10.12998/wjcc.v9.i4.886

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Isolated interrupted aortic arch in an adult: A case report

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World J Clin Cases. 2021 Feb 6;9(4):992-998. doi: 10.12998/wjcc.v9.i4.992.

ABSTRACT

BACKGROUND: Interrupted aortic arch (IAA) is a rare congenital heart disease defined by an interruption of the lumen and anatomical continuity between the ascending and descending major arteries. It is usually found within a few hours or days of birth. Without surgery, the chances of survival are low. If IAA patients have an effective collateral circulation established, they can survive into adulthood. However, IAA in adults is extremely rare, with few reported cases.

CASE SUMMARY: A 27-year-old woman presented with a 6-year history of progressively worsening shortness of breath and chest tightness on exertion. She had cyanotic lips and clubbing of the fingers. A transthoracic echocardiogram revealed an enlarged heart and dilation of the main pulmonary artery. There was an abnormal 9 mm passage between the descending aorta and pulmonary artery. Th e ventricular septal outflow tract had a 14 mm defect. Doppler ultrasound suggested a patent ductus arteriosus and computed tomographic angiography showed the absence of the aortic arch. The diagnoses were ventricular septal defect, patent ductus arteriosus, and definite interruption of the aortic arch. Although surgical correction was recommended, the patient declined due to the surgical risks and was treated with medications to reduce pulmonary artery pressure and treat heart failure. Her condition has been stable for 12 mo of follow-up.

CONCLUSION: Although rare, IAA should be considered in adults with refractory hypertension or unexplained congestive heart failure.

PMID:33585649 | PMC:PMC7852638 | DOI:10.12998/wjcc.v9.i4.992

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Open reduction and Herbert screw fixation of Pipkin type IV femoral head fracture in an adolescent: A case report

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World J Clin Cases. 2021 Feb 6;9(4):898-903. doi: 10.12998/wjcc.v9.i4.898.

ABSTRACT

BACKGROUND: Femoral head fracture is extremely rare in children. This may be the youngest patient with femoral head fracture ever reported in the literature. There are few pediatric studies that focus on cases treated with open reduction via the modified Hardinge approach.

CASE SUMMARY: A 14-year-old female adolescent suffered a serious traffic accident when she was sitting on the back seat of a motorcycle. A pelvic radiograph and computed tomography revealed a proximal femoral fracture and slight acetabular rim fracture. This was diagnosed as a Pipkin type IV femoral head fracture. An open reduction and Herbert screw fixation was performed via a modified Hardinge approach. After 1-year follow-up, the patient could walk without aid and participate in physical activities. The X-ray results showed that the fractures healed well with no evidence of complications.

CONCLUSION: Open reduction and Herbert screw fixation is an available therapy to treat Pipkin type IV femoral head fractures in children.

PMID:33585637 | PMC:PMC7852633 | DOI:10.12998/wjcc.v9.i4.898

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Effects of different statins application methods on plaques in patients with coronary atherosclerosis

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World J Clin Cases. 2021 Feb 6;9(4):812-821. doi: 10.12998/wjcc.v9.i4.812.

ABSTRACT

BACKGROUND: Discontinued application of statins may be related to adverse cardiovascular events. However, it is unclear whether different statins administration methods have effects on coronary artery plaques.

AIM: To evaluate the effects of different statins application methods on plaques in patients with coronary atherosclerosis.

METHODS: A total of 100 patients diagnosed with atherosclerotic plaque by coronary artery computed tomography were continuously selected and divided into three groups according to different statins administration methods (discontinued application group, n = 32; intermittent application group, n = 39; sustained application group, n = 29). The effects of the different statins application methods on coronary atherosclerotic plaque were assessed.

RESULTS: The volume change and rate of change of the most severe plaques were significantly reduced in the sustained application group (P ≤ 0.001). The volume change of the most severe plaques correlated positively with low-density lipoprotein (LDL-C) levels only in the sustained application group (R = 0.362, P = 0.013). There were no changes in plaques or LDL-C levels in the intermittent and discontinued application groups.

CONCLUSION: Continuous application of statins is effective for controlling plaque progression, whereas discontinued or intermittent administration of statins is not conducive to controlling plaques. Only with continuous statins administration can a reduction in LDL-C levels result in plaque volume shrinkage.

PMID:33585627 | PMC:PMC7852639 | DOI:10.12998/wjcc.v9.i4.812

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Acute pancreatitis with pulmonary embolism: A case report

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World J Clin Cases. 2021 Feb 6;9(4):904-911. doi: 10.12998/wjcc.v9.i4.904.

ABSTRACT

BACKGROUND: Acute pancreatitis (AP) is a common critical disease of the digestive system that is often associated with multiple complications. Vascular complications are relatively rare and are one of the causes of death. AP complicated with pulmonary embolism (PE) is even rarer, and there are no reports of AP complicated with PE in elderly patients.

CASE SUMMARY: We describe a rare case of AP complicated with PE and review the literature. A 68-year-old woman was diagnosed with AP due to widespread abdominal pain. During the course of treatment, the patient had shortness of breath and progressively worsening dyspnea without chest pain or hemoptysis with a progressive increase in D-dimer and fibrin degradation product. Respiratory failure and right heart failure occurred, and refractory hypoxemia remained after mechanical ventilation. Plain chest c omputed tomography revealed a small amount of left pleural effusion and external pressure atelectasis in the lower lobe of the left lung but no findings that could lead to refractory hypoxemia. Color Doppler ultrasound indicated pulmonary hypertension and extensive venous thrombosis in the lower extremities. Chest computed tomography angiography finally suggested pulmonary thromboembolism. The patient's dyspnea symptoms disappeared after anticoagulation treatment.

CONCLUSION: During the diagnosis and treatment of AP, it is necessary to dynamically monitor D-dimer and consider PE.

PMID:33585638 | PMC:PMC7852627 | DOI:10.12998/wjcc.v9.i4.904

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Reciprocal hematogenous osteomyelitis of the femurs caused by Anaerococcus prevotii: A case report

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World J Clin Cases. 2021 Feb 6;9(4):830-837. doi: 10.12998/wjcc.v9.i4.830.

ABSTRACT

BACKGROUND: Haematogenous osteomyelitis is an extremely rare disease occurring in adults, especially in developed countries. It is clearly a systemic infection, because bacteraemia spreads over proximal and distal long bones or paravertebral plexuses, resulting in acute or chronic bone infection and destruction.

CASE SUMMARY: A 46-year-old Caucasian male was complaining of a left thigh pain. It is known from the anamnesis that the patient developed severe pneumonia three months ago before the onset of these symptoms. The patient was diagnosed with haematogenous osteomyelitis, which developed a turbulent course and required complex combination therapy. The primary pathogen is thought to be Anaerococcus prevotii, which caused pneumonia before the onset of signs of osteomyelitis. Unfortunately, due to the complexity of identifying anaerobes an d contributing nosocomial infections, the primary pathogen was not extracted immediately. After the manifestation of this disease, pathological fractures occurred in both femurs, as well as purulent processes in the lungs and molars accompanied. The patient received broad-spectrum antibiotic therapy and countless amounts of orthopaedic and reconstructive surgeries, but no positive effect was observed. The patient underwent osteosynthesis using an Ilizarov's external fixation apparatus, re-fixations, external AO, debridements, intrame-dullary osteosynthesis with a silver-coated intramedullary nail, abscessotomies. The right femur healed completely after the pathological fracture and osteomyelitis did not recur. Left femur could not be saved due to non-healing, knee contracture and bone destruction. After almost three years of struggle, it was decided to amputate the left limb, after which the signs of osteomyelitis no longer appeared.

CONCLUSION: To sum it all up, complicated o r chronic osteomyelitis requires surgery to remove the infected tissue and bone. Osteomyelitis surgery prevents the infection from spreading further or getting even worse up to such condition that amputation is the only option left.

PMID:33585629 | PMC:PMC7852651 | DOI:10.12998/wjcc.v9.i4.830

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Apert syndrome diagnosed by prenatal ultrasound combined with magnetic resonance imaging and whole exome sequencing: A case report

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World J Clin Cases. 2021 Feb 6;9(4):912-918. doi: 10.12998/wjcc.v9.i4.912.

ABSTRACT

BACKGROUND: Most cases of Apert syndrome (AS) are found after birth. Cases of AS diagnosed by ultrasound combined with magnetic resonance imaging (MRI) and whole exome sequencing (WES) during pregnancy are rare.

CASE SUMMARY: We present the case of a 34-year old female patient (gravida 2, para 1) whose fetus was diagnosed with AS during pregnancy. Fetal ultrasound performed at 30, 2/7 wk of pregnancy showed abnormalities. MRI and three-dimensional ultrasound performed at 31, 1/7 wk of pregnancy showed the possibility of AS. Chromosome examination and core family WES were conducted at 31, 5/7 wk of pregnancy. The results showed that FGFR2 in the fetus had a c.755C>G missense mutation in its nucleotide, and AS was confirmed.

CONCLUSION: This case highlights the importance of imaging examinations. Prenatal ultrasound combined with MRI can identify fetal morphological abnormalities accurately, which can be confirmed by WES.

PMID:33585639 | PMC:PMC7852645 | DOI:10.12998/wjcc.v9.i4.912

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