Outcomes and Incidence of PF-ILD According to Different Definitions in a Real-World Setting

Background: Almost one-third of fibrosing ILD (fILDs) have a clinical disease behavior similar to IPF, demonstrating a progressive phenotype (PF-ILD). However, there are no globally accepted criteria on the definition of a progressive phenotype in non-IPF fILD yet. Four different definitions have been used; however, no internationally accepted definition currently exists.Research Question: To compare the clinical and functional characteristics of progressive fILD according to the currently available definitions.Study design and methods: Cases of fILD were identified retrospectively from the database of the tertiary referral center for ILD in Heidelberg. Lung function, clinical signs of progression, and radiological changes were evaluated. Patients with fILD were considered to have progression according to each of the four available definitions: Cottin (CO), RELIEF (RE), INBUILD (IN), and UILD study. Lung function changes, expressed as mean absolute decline of FVC%, were reported every 3 months following diagnosis and analyzed in the context of each definition. Survival was also analyzed.Results: A total of 566 patients with non-IPF fILD were included in the analysis. Applying CO-, RE-, IN-, and UILD-definitions, 232 (41%), 183 (32%), 274 (48%), and 174 (31%) patients were defined as PF-ILD, respectively. RE- and UILD-criteria were the most stringent, with only 32 and 31% patients defined as progressive, while IN- was the most broad, with almost 50% of patients defined as progressive. CO- definition was in-between, classifying 41% as progressive. PF ILD patients with a UILD definition had worse prognosis.Interpretation: Depending on the definition used, the existing criteria identify different groups of patients with progressive fILD, and this may have important prognostic and therapeutic implications.

Observational study of clinico-radiological follow-up of COVID-19 pneumonia: a district general hospital experience in the UK

Background The British Thoracic Society (BTS) recommends that all patients admitted with COVID-19 pneumonia should have a chest X-ray (CXR) and clinical follow-up at 6 or 12 weeks, depending on the disease severity. Little data is available on long-term CXR follow-up for moderate and severe COVID-19 pneumonia. This study aims to evaluate compliance with clinico-radiological follow-up of patients recovering from COVID-19 pneumonia at a local hospital in the UK, as per the BTS guidance, and to analyse radiological changes at clinical follow-up at 12 weeks, in order to risk-stratify and improve patient outcomes. Methods This is a single-centre retrospective audit of 255 consecutive COVID-19 positive patients admitted to a local hospital in the UK over 5 months between May and October 2020. All CXRs and clinic follow-up at 12 ± 8 weeks were checked on an electronic database. Results Over one in two (131/255) patients had CXR evidence of COVID-19 pneumonia during the initial hospital admission. Half of the patients (60/131) died before CXR or clinic follow-up. Fifty-eight percent (41/71) of the surviving patients had a follow-up CXR, and only two developed respiratory complications- one had residual lung fibrosis, another a pulmonary embolism. Eighty-eight percent (36/41) of the patients had either resolution or improved radiological changes at follow-up. Most patients who had abnormal follow-up CXR were symptomatic (6/8), and many asymptomatic patients at follow-up had a normal CXR (10/12). Conclusions Although there were concerns about interstitial lung disease (ILD) incidence in patients with COVID-19 pneumonia, most of our patients with COVID-19 pneumonia had no pulmonary complications at follow-up with CXR. This emphasises that CXR, a cost-effective investigation, can be used to risk-stratify patients for long term pulmonary complications following their COVID-19 pneumonia. However, we acknowledge the limitations of a low CXR and clinic follow-up rate in our cohort.

Mortality of covid19 in sickle cell disease systematic review

The covid19 infection is pandemic disease are more commonly in chronic diseases. As covid19 case pulmonary infection, the current study focuses on sickle cell disease patients are suitable to acute chest syndrome. It will clarify the risk factors for covid19 infection and its clinical features in sickle cell disease. It also reviews the radiological findings to gain more data on this pandemic disease. The current study includes 44 cases of sickle cell disease having covid19 infection. The entire cases include sickle cell disease only in 89% whereas the other sickle cell disease cases are coexisted with beta- thalassemia in 9.1%. The fourth decade is the highest peak incidence. There are several complications found to be in sickle cell disease are acute chest syndrome, chronic leg ulcer, renal failure exceeding 10%. The bronchial asthma found to be in 6.9% and avascular necrosis found to be 11.4% whereas the cerebrovascular accident found to be in 13.8%. The radiological changes include lung tissues, alveoli, pleural cavities and pulmonary arteries in different rate. The incidence of recovery found to be in 93% whereas the death found to be in 7%. It found to be involving in respiratory and gastrointestinal systems result in different clinical features in different rate. This study compares the clinical features, findings investigation and complications between sex and decades. Further, this study clarifies recovery and mortality rate between sex and decades. Knowing and understanding covid19 infection in sickle cell disease, physicians will be able to provide high quality of medical services.

Systematic Review of Cerebral Phenotypes Associated with Monogenic Cerebral Small Vessel Disease

Background: Cerebral small vessel disease (cSVD) is an important cause of stroke and vascular dementia. Most cases are multifactorial, but an emerging minority have a monogenic cause. While NOTCH3 is the best-known gene, several others have been reported. We aimed to summarise the cerebral phenotypes associated with these more recent cSVD genes. Methods: We performed a systematic review (PROSPERO: CRD42020196720), searching Medline/Embase (conception to July 2020) for any language publications describing COL4A1/2, TREX1, HTRA1, ADA2, or CTSA pathogenic variant carriers. We extracted data about individuals characteristics, clinical and vascular radiological cerebral phenotypes. We summarised phenotype frequencies per gene, comparing patterns across genes. Results: We screened 6,485 publications including 402, and extracted data on 390 COL4A1, 123 TREX1, 44 HTRA1 homozygous, 41 COL4A2, 346 ADA2, 82 HTRA1 heterozygous, and 14 CTSA individuals. Mean age ranged from 15 (ADA2) to 59 years (HTRA1 heterozygotes). Clinical phenotype frequencies varied widely: stroke 9% (TREX1) to 52% (HTRA1 heterozygotes), cognitive features 0% (ADA2) to 64% (HTRA1 homozygotes), psychiatric features 0% (COL4A2; ADA2) to 57% (CTSA). Among individuals with neuroimaging, vascular radiological phenotypes appeared common, ranging from 62% (ADA2) to 100% (HTRA1 homozygotes; CTSA). White matter lesions were the most common pathology, except in ADA2 and COL4A2 cases, where ischaemic and haemorrhagic lesions dominated, respectively. Conclusions: There appear to be differences in cerebral manifestations across cSVD genes. Vascular radiological changes were more common than clinical neurological phenotypes, and present in the majority of individuals with reported neuroimaging. However, these results may be affected by age and biases inherent to case reports. In the future, better characterisation of associated phenotypes, as well as insights from population-based studies, should improve our understanding of monogenic cSVD to inform genetic testing, guide clinical management, and help unravel underlying disease mechanisms.

Radiological changes, infections and neurological complications after reverse shoulder arthroplasty related to different design types and their rates: Part II

Early reported complication rates with the Grammont-type reverse shoulder arthroplasty (RSA) were very high, up to 24%. A ‘problem’ is defined as an intraoperative or postoperative event that is not likely to affect the patient’s final outcome, such as intraoperative cement extravasation and radiographic changes. A ‘complication’ is defined as an intraoperative or postoperative event that is likely to affect the patient’s final outcome, including infection, neurologic injury and intrathoracic central glenoid screw placement. Radiographic changes around the glenoid or humeral components of the RSA are very frequently observed and described in the literature. High complication rates related to the Grammont RSA design led to development of non-Grammont designs which led to a dramatic fall in the majority of complications. The percentage of radiological changes after RSA is not negligible and remains unsolved, despite a decrease in its occurrence in the last decade. However, such changes should be now considered as simple problems because they rarely have a negative influence on the patient’s final outcome, and their prevalence has dramatically decreased. With further changes in indications and designs for RSA, it is crucial to accurately track the rates and types of complications to justify its new designs and increased indications. Cite this article: EFORT Open Rev 2021;6:1109-1121. DOI: 10.1302/2058-5241.6.210040

Unilateral adrenal infarction in pregnancy with associated acute hypoadrenalism and subsequent spontaneous biochemical and radiological resolution

Authors describe a rare case of acute adrenal infarction in pregnancy resulting in hypoadrenalism requiring hydrocortisone replacement therapy and anticoagulation with subsequent resolution of the radiological changes and complete recovery of the glucocorticoid reserve during clinical follow-up.

Rhizomelic limb shortness and abnormal facies: short stature syndrome sharing some manifestations with robinow syndrome

In children, short stature and disproportionate skeletal growth are associated with many syndromes and these syndromes have characteristic radiological changes which help in their diagnosis. However, many of them have no well-defined diagnostic criteria, and thus overlap between apparently distinct syndromes is common. Here, we report a child with characteristic features seen distinct from any other dwarfing syndrome known to us with some of the features similar to Robinow syndrome. Newborn had a broad and prominent forehead, mid-face hypoplasia, hypertelorism, flat nasal bridge, upturned nose, elongated philtrum, protruding tongue, single crease in the left hand, widely spaced nipple, brachydactyly in lower limbs, and disproportionately short rhizomelic upper and lower limbs. Based on these features, a diagnosis of Robinow syndrome was made. The case was the first of his kind admitted to our institute. The diagnosis was made clinically after follow-up.

Epidemiological characteristics, clinical characteristics, and prognostic factors of children with atopy hospitalised with adenovirus pneumonia

Background Atopy may be associated with disease severity and a poor prognosis of human adenovirus (HAdV) pneumonia in children. Our aim was to observe the clinical characteristics and pulmonary radiological changes in children with atopy and HAdV pneumonia in China. Methods Children hospitalised with HAdV pneumonia from June 2018 to December 2019 were analysed. All children were divided into atopic with HAdV, non-atopic with HAdV, and atopic without HAdV infection group. Each group was further divided into the mild and severe pneumonia groups according to disease severity. Standard treatment was initiated after admission, and regular follow-up evaluations were conducted at 1 month after discharge. Baseline and clinical characteristics and pulmonary radiological changes in children with and without atopy were evaluated. Risk factors associated with small airway lesions in patients with HAdV pneumonia were analysed. Results The eosinophil count in the atopic group was significantly higher than that in the non-atopic group (P < 0.05). Severe coughing, wheezing, and small airway lesions on chest high-resolution computed tomography (HRCT) upon admission, after discharge and 1 month after discharge were significantly higher in the atopic group (with or without HAdV infection) than in the non-atopic group (P < 0.05). There were significant differences in the number of patients with wheezing and small airway lesions during hospitalisation and after discharge among the three groups (P < 0.05). The risks of small airway lesions in children with a family or personal history of asthma, severe infection, atopy, and HAdV infection were 2.1-, 2.7-, 1.9-, 2.1-, and 1.4-times higher than those in children without these characteristics, respectively. Conclusions Children with atopy and HAdV pneumonia may experience severe coughing in mild cases and wheezing in mild and severe cases. Children with atopy are more susceptible to the development of small airway lesions, recurrent wheezing after discharge and slower recovery of small airway lesions as observed on pulmonary imaging than non-atopic children after HAdV infection. A family or personal history of asthma, atopy, severe infection, and HAdV infection are independent risk factors associated with the development of small airway lesion as observed on chest HRCT.