Multidetector CT findings of primary pleural angiosarcoma : a systematic review, an additional cases report

Background To demonstrate and analyze the relatively common imaging findings in this rare primary pleural angiosarcoma (PPA). Case presentation Three cases of PPA, proven by video-assisted thoracic surgery biopsies are retrospectively reviewed. Patients were all male. Age ranges from 65 to 75 years old age (mean; 69). Major chief complaints were dyspnea and chest pain. One has a history of colon cancer, the other has a tuberculosis history and the other has no known history. Multidetector chest CT and PET CT were all done. Immunohistochemical studies were performed including CD31, CD34, or factor VIII-related antigen, vimentin, and cytokeratin. We also review the literatures on recently published PPA. All masses were from 1 to 10 cm. All three patients had multiple pleural based masses, which were ovoid in shape with relatively sharp margin in unilateral hemithorax. Multiple small circumscribed pleural masses are limited in the pleural space in two patients, whereas two, huge lobulated masses about up to 10 cm were present with pleural and extrapleural involvement in one patient. In two patients with pleural mass only, multiple pleural masses were only seen in parietal pleura in one patient and were in both visceral and parietal pleura in one patient. Pleural effusion were found in one side in one patient and in both sides in one patient. One angiosarcoma was arised from chronic tuberculotic pleurisy sequelae. All pleural masses are heterogenous with irregular internal low densities in all patients. Hematogenous metastases were found in liver, vertebra, rib in one patient, and were in lungs with mediastinal lymph node metastases in the other patient. Three patients survived for longer than 3months after diagnosis, but continued to deteriorate rapidly. Two patients underwent chemotherapy after surgical excision, and the other one with multiple metastases treated chemotherapy after CT-guided biopsy, but eventually all died. As a result of comparative analysis of a total of 13 patients’ images including 10 cases previously published, there was pleural effusion in all except 2 cases. Conclusions PPA were all necrotic without any vascularized enhancing nature, and manifested as unilateral circumscribed or localized pleural-based masses.

Osteopetrosis in a Patient of Systemic Sclerosis Sine Scleroderma: A Rare Association

Systemic sclerosis is a connective tissue disorder of unknown etiology. Although it is a multisystemic disorder, skin thickening is considered as a hallmark of the disease. It usually involves the lungs, gastrointestinal, and musculoskeletal systems. However, a rare subset of systemic sclerosis, systemic sclerosis sine scleroderma, is characterized by internal organ involvement and positive serologic markers with the total or partial absence of cutaneous manifestations. We present a rare association of osteopetrosis in a case of systemic sclerosis sine scleroderma, in a 22-year-old male patient, who presented with pulmonary symptoms as his chief complaints, unreported so far in literature.

Uncommon cause of respiratory failure due to a bezoar in the hypopharynx: a case report

Background Trichotillomania and trichophagia cause trichobezoars, which are masses made of hair. The main presentation of this condition is abdominal pain. However, other complications include gastric outlet obstruction, nausea, vomiting, weight loss, malnutrition, hematemesis, diarrhea, and constipation. Case presentation A 57-year-old woman with trichotillomania was admitted to the Emergency Department with the chief complaints of dyspnea on exertion, shortness of breath, dysphagia, generalized weakness, and hoarseness. Spiral chest computed tomography (CT) scan did not reveal any parenchymal lesions Pulmonary CT angiography did not reveal pulmonary embolism. The patient was admitted to the Surgery Department for hand fasciotomy due to contrast leakage, and during laryngoscopy, a trichobezoar was detected that was removed with Magill forceps. Conclusions Rare cases of trichobezoars can be observed in humans with gastrointestinal and respiratory symptoms. Precise and timely diagnosis are key for the prevention of more invasive diagnostic procedures.

Two different presentations of de novo variants of CSNK2B: two case reports

Background Poirier–Bienvenu neurodevelopmental syndrome is a neurologic disorder caused by mutations in the CSNK2B gene. It is mostly characterized by early-onset seizures, hypotonia, and mild dysmorphic features. Craniodigital syndrome is a recently described disorder also related to CSNK2B, with a single report in the literature. Objective To report two unrelated cases of children harboring CSNK2B variants (NM_001320.6) who presented with distinct diseases. Case report Case 1 is a 7-month-old, Caucasian, female patient with chief complaints of severe hypotonia and drug-refractory myoclonic epilepsy, with a likely pathogenic de novo variant c.494A>G (p.His165Arg). Case 2 is a 5-year-old male, Latino patient with craniodigital intellectual disability syndrome subjacent to a de novo, likely pathogenic variant c.94G>T (p.Asp32Tyr). His dysmorphic features included facial dysmorphisms, supernumerary nipples, and left-hand postaxial polydactyly. Conclusion This report suggest that the CSNK2B gene may be involved in the physiopathology of neurodevelopmental disorders and variable dysmorphic features.

Study of Clinical Features and Diagnosis Pattern of Duchene Muscular Dystrophy in Southern India

Background Duchene muscular dystrophy (DMD) is an X-linked progressive muscle disorder that is characterized by proximal muscle weakness followed by a premature death in young boys. There is a low index of reports on diagnosis ratio and clinical features in Southern India. Objective The present study aimed to conduct an observational survey on preliminary analysis, family history, associated complaints, and diagnosis ratio of DMD in southern regions of India. Materials and Methods A systematic observation and survey were conducted on clinically confirmed DMD patients registered between 2019 and 2021 through the questionnaire. The questionnaire and pattern of study were identified by exploring published and unpublished studies available from electronic databases and critical assessment criteria considered by physicians. Preliminary analysis such as onset criteria, motor difficulties, milestone delay; family history and consanguinity analysis; chief complaints (ambulatory status, lordosis, respiratory, and cardiac outcomes), associated complaints such as enlarged tongue, oral hygiene, behavioral problems; and other similar parameters were studied. An assessment of the diagnosis rate and pattern was performed. Statistical analysis The data were reviewed and interpreted through statistical methods mean ± standard deviation represented as a percentage. Results In total, 400 DMD patients were included and 250 participated in the study. The onset age group was 2 to 5 years in 37% of the population. Milestone delay was seen in 86%; consanguinity marriage of parents was reported in 39%. Frequent falls were reported in 62% in 5 to 8 years old group. Wheelchair status was reported in 65% in 9 to 12 years old. Cervical and lumbar lordoses were seen in 57 and 69%, respectively, in above 13 years old. Respiratory and cardiac complications were 88 and 78% reported in above 13 years old, respectively. Other major associated complaints such as enlarged tongue were reported in 79%. Fifty-one percent underwent genetic diagnosis and 79% of the population underwent serum creatine phosphokinase (CPK) analysis for the confirmation of DMD. Conclusion In this study population of South India, milestone delay was a major observation. Although there was a slight margin, family history shows “no blood relation among parents” in the majority of the study population. Chief complaints were predominantly severe above 13-year age group population. Serum CPK was the first choice for the first investigation, which is followed by a genetic diagnosis.

Nonspecific abdominal pain: Do I have to allocate a specific diagnosis for every patient?

Acute abdominal pain is one of the most common chief complaints in the acute setting all over the world. The definitive diagnoses of these patients vary depending on age, gender, geographical and sociodemographic characteristics etc. Apart from these, aging of the population, obesity, advanced diagnostic imaging modalities and changes in nutritional habits also have an impact on the rates of specific diagnoses. On the other hand, nonspecific abdominal pain constitutes almost half of all visits due to acute abdominal pain in the acute care setting. This phenomenon is the main differential diagnostic problem in the management of these patients because missing a life-threatening condition can cause enormous medicolegal problems for the caregivers. Certain diagnostic scoring systems have also been developed to facilitate recognition and management of these conditions. This article aims to review the entity and underline the challenges it can bring to the scene.

The Effect of the COVID-19 Pandemic on the Pediatric Emergency Department Flow

ABSTRACT Objective: To determine how the early stages of the COVID-19 pandemic affected the utilization of the Pediatric Emergency Department (PED). Methods: Cross-sectional study of PED visits during January through April, 2016-2020. Data included: total PED visits, emergency severity index (ESI), disposition, chief complaint, age (months), time from first Provider to Disposition (PTD), and PED length of stay (PED-LOS). P-value <0.01 was statistically significant. Results: In total, 67,499 visits were reported. There was a significant decrease in PED visits of 24-71% from March to April 2020. Chief complaints for fever and cough were highest in March 2020; while April 2020 had a shorter mean PED-LOS (from 158 to 123 minutes), an increase of admissions (from 8% to 14%), a decrease in ESI 4 (10%), and an increase in ESI 3 (8%) (p<0.001). There was no difference in mean monthly PTD time. Conclusions: Patient flow in the PED was negatively affected by a decrease in PED visits and increase in admission rate that may be related to higher acuity. By understanding the interaction between hospital processes on PEDs and patient factors during a pandemic, we are able to anticipate and better allocate future resources.

Case Report Onventricular Septal Defect (VSD) with Pulmonary Hypertention (Eisenmenger Syndrome) with LSCS

Background: A ventricular septal defect is a defect of the ventricular septal wall where the abnormal opening is present in between the two ventricles. Eisenmenger syndrome is a condition it may develop due to unrepaired heart defect and complication of unrepaired heart defect who is born congenitally. The congenital heart defects lead to Eisenmenger syndrome it developed due to abnormal blood circulate throughout the heart and lungs. Case Report: The 23-year-old female was admitted to AVBRH with the chief complaints of a history of amenorrhoea since 8 months, complaints of breathlessness during exertion since the 7th month of pregnancy and she was referred to AVBRH from Amravati on date 27/1/2021. At the age of 14 years, she had a history of cyanosis at the time of fever and had recurrent episodes of fever for 2-3 yrs, lasting for 2-3 weeks. She is primigravida and had undergone LSCS on date 3/2/2021. She delivered a male child on date 3/2/2021 of birth weight 1.9kg at 12:15 pm. Discussion: Patient with pulmonary hypertension and Eisenmenger syndrome where the surgery is contraindicated because in Eisenmenger syndrome surgery is life-threatening. Only medical management is available to treat Eisenmenger syndrome. Conclusion: Ventricular septal defect in adult females where the pregnancy is contraindicated. Epidural anesthesia should be preferred while the patient undergoes the lower segment cesarean section. This case report concludes that in this condition emergency LSCS is the first choice of treatment and oxygen therapy.

A Case Report of Steven Johnson Syndrome

Introduction: In 1922, two doctors, Albert Mason Stevens and Frank Johnson, examined purulent conjunctivitis.” Background: Stevens-Johnson syndrome was named after them as a result of their study. The incidence rate is 7 cases per million populations per year. Case Presentation: Master Yash Ghudam was brought to AVBRH by his parents with chief complaints of fever since 5 days and erythematous lesions all over body since 3 days. History of present illness: Patient was apparently alright 5 days back, and then he started having fever which was of high grade and was not associated with chills and rigor. Patient was treated on OPD basis and the symptoms of an unexplained disease in two young boys, aged 7 and 8, who had "an unusual, generalised eruption of continued fever, inflamed buccal mucosa, and extreme some antibiotic was given, but there was no relief, after 2 days there was ulcers formation inside the mouth for which some ointment and syrup becosule was started. But lesions were increasing. 3 days back the lesions first appeared on chest then got spread to legs and hands. For which patient was admitted in Chandrapur hospital from were the patient was referred to AVBRH for further management. Interventions: The patient was treated the patient was started on intravenous and orally Cortecosteroids, Omnacortil 10mg, Antibiotics- Inj. Ceftriaxone1gm IV 12 hourly [100mg/kg/day], inj. Amikacin 150mg IV 12 hourly [15mg/kg/day], Syp. Mucaine gel 2tsp BD – swish and swallow), Syp. Cital 2.5ml TDS, Tab. Chymoral Forte TDS, Inj. Pantop 20mg IV 24 hourly (1mg/kg/dose). Pandya’s Formula: Syp. Gelusil 5ml, Syp. Benadryl 5ml, Syp. Omnacortil 5ml. Skin allograft: It has been planned. Conclusion: In this study, we mainly focus on medical management and outstanding nursing care helped prevent farther complication. Overall, the patient's reaction was positive, though recovery time from Steven johnson syndrome varies from person to person, taking weeks, months, or even years. However, only a small number of people completely recover, while some have long-term consequences. She took a long time to get back on her feet.

Guillain Barre Syndrome: A Case Report and Literature Review

Introduction: The arrival of Guillain-Barre Syndrome is sudden. It’s a type of neuropathy caused by the immune system. Nutrition is very crucial. In impoverished countries, it is a disabling disease. Autoantibodies against diverse antigens can be seen in the outlying site. The occurrence links 0.4 to 1.7 million individuals per year. Case Presentation: A 5-year-old boy was taken to the hospital with chief complaints of Weakness in bilateral upper and lower limbs, trouble in swallowing, inability to hold the neck, mouth-frothing, fever spikes. On physical examination, the patient has experienced weakness in bilateral upper and lower limbs, Bulbar weakness is present, pain experienced in both legs, the gag reflex is absent, In Cardiovascular System, S1 and S2 sound are present, In Respiratory System, Air entry is bilaterally equal, pupils are reflected light, tone, and power of upper and lower limbs are decreased, then treatment was started as soon as possible, he has not improved after receiving treatment, and the patient is on ventilator support, with treatment continuing until the end of my care. Conclusion: In this study, we primarily focus on professional management, and outstanding nursing care may give the holistic care that Guillain Barre Syndrome requires while also effectively managing the challenging case. The comprehensive health care team collaborates to help the patient achieve their prior level of independence and satisfaction after a full recovery.