Unveiling the complexity of right middle lobe syndrome: a case series highlighting the association with asthma.

Introduction and importance: Right middle lobe syndrome (MLS) is a rare lung disorder primarily affecting children with a history of asthma or atopy. It encompasses a range of pathological and clinical conditions, from recurrent collapses of the middle lobe to bronchiectasis. In this study, the authors present a case series featuring four individuals with MLS associated with asthma, aiming to deepen our understanding of this uncommon condition. Case presentation: Four paediatric patients with right MLS exhibited symptoms of persistent cough, dyspnoea, and recurrent asthma exacerbations. Radiographic evaluations confirmed features consistent with right MLS, and bronchoscopy revealed mucus plugs and oedematous airways obstructing the right middle lobe bronchus. Treatment with bronchodilators, antibiotics, and corticosteroids led to symptom improvement and resolution of atelectasis. Clinical discussion: MLS is a rare condition characterized by chronic collapse of the right middle lobe and bronchiectasis. It is challenging to diagnose MLS, but computed tomography (CT) scans provide detailed lung images for confirmation. Treatment focuses on addressing the underlying cause, such as infections or mucus obstruction. Lobectomy may be considered in severe cases. This case series emphasizes the need for further research on MLS, as its rarity and characteristics remain unclear. Conclusion: These cases exhibited obstructive MLS with and without asthma. Accurate diagnosis is challenging, requiring imaging techniques. MLS has clinical implications, particularly in asthma patients. Future studies should focus on understanding the aetiology of non-obstructive MLS.

Clinical presentation and management of multisystem inflammatory syndrome in children associated with covid-19: a retrospective observational descriptive study in a pediatric hospital in Syria.

OBJECTIVE: Multisystem Inflammatory Syndrome in Children (MIS-C) associated with COVID-19 is a rare and serious medical condition. This study aims to review the clinical presentation, laboratory parameters, outcomes, and management of MIS-C cases in a pediatric hospital in Syria. METHODS: This retrospective observational study aimed to investigate MIS-C between May 2020 and October 2021. Data collection involved extracting information from medical records, and patients were identified based on the case definition established by the World Health Organization (WHO). Various laboratory investigations, diagnostic evaluations, clinical presentations, and treatments were performed to assess patients. Descriptive statistical analysis was conducted using Microsoft Excel. RESULTS: A total of 232 COVID-19 cases were reported with COVID-19 Infection. Among these cases, 25 (10.77%) were identified as MIS-C. The median age of the patients was 5.5 years, with the majority being male patients (72%). Patients experienced fever (100%), bilateral conjunctivitis (88%), rash (84%), gastrointestinal symptoms (76%), and cardiac dysfunction (72%). Other notable findings included oral cavity changes (64%), edema (36%), cervical lymphadenopathy (36%), and neurological manifestations (28%). Respiratory symptoms were uncommon (16%). All patients recovered, with no recorded deaths. CONCLUSION: The predominant presence of positive SARS-CoV-2 IgG in the majority of patients in this study supports the post-infectious nature of MIS-C. Respiratory symptoms were less prevalent in both pediatric COVID-19 and MIS-C patients. Early supportive care is crucial in management, although additional research is needed to establish definitive guidelines. Larger studies are necessary to overcome the limitations of this study and to enhance our understanding of MIS-C in pediatric COVID-19 patients.

Autoimmune hemolytic anemia associated with COVID-19 infection: a rare case report.

Coronavirus disease 2019 (COVID-19) is an illness due to severe acute respiratory syndrome, symptoms and severity of disease varies from patient to patient, autoimmune hemolytic anemia (AIHA) in children with COVID-19 is rare. Case presentation: A 12-year-old female presented with fever, headache, myalgia, and hematuria. At admission, she was hemodynamically stable, severe anemia was present, and severe acute respiratory syndrome coronavirus 2 infection was confirmed by RT-PCR. The diagnosis of AIHA was confirmed and treated. Discussion: There are few reports of patients with AIHA and COVID-19. However, the majority of patients in these reports also have autoantibodies and other underlying conditions known to be associated with the development of AIHA. Conclusion: In this current pandemic, it should be taken into account that previously healthy children with severe acute respiratory syndrome coronavirus 2 infection have been found to have severe hemolytic anemia in the absence of COVID-19.

Recurrent pulmonary infection leads to the diagnosis of triple A syndrome: a case report.

INTRODUCTION: Triple A syndrome is a very uncommon disease marked by a triad of adrenocorticotrophic hormone (ACTH)-resistant features: adrenal insufficiency, alacrimia, and achalasia. It presents in several clinical forms with undetermined incidence and shows an autosomal pattern of inheritance. It is caused by a variety of mutations in the AAAS genes which encode a protein of unknown function called ALADIN. Diagnosis depends on clinical manifestations, laboratory test results, imaging and endoscopic findings, and Schirmer's test. The treatment includes artificial tears, glucocorticoid replacement therapy, and treatment of achalasia. CASE PRESENTATION: A 12-year-old Syrian girl was referred to Damascus University's Children's Hospital for recurrent pulmonary infection. Her mother had noted an absence of tears when crying since birth, diffused pigmentations since birth, especially on the cheeks and genitals, recurrent vomiting of both solid and liquid foods, and recurrent exacerbations of bronchitis and recurrent pneumonia. ACTH and blood cortisol levels indicated an adrenal insufficiency, chest computed tomography and barium swallow test results indicated achalasia, tear break-up time as well as eye examination indicated alacrimia, which led to the diagnosis of triple A syndrome. Treatment included Heller cardiomyotomy, artificial tears, and hydrocortisone (15-30 mg/m2), as well as continuous observation of ACTH levels. DISCUSSION: Triple A syndrome (which is characterized by the triad of achalasia, alacrima, adrenal insufficiency) is a rare multisystem disease. It has a genetic background and is potentially fatal. This syndrome is often misdiagnosed, especially in regions where it is expected to have a high prevalence rate (regions with documented cases and high rate of consanguinous marriage), This study is the first documentation of triple A syndrome in Syria, a country where consanguineous marriage is common. This syndrome should be kept in mind when a child presents with one or more of its characteristic features.

Congenital lobar emphysema case report: A frequently misdiagnosed disease.

Introduction and importance: Congenital Lobar Emphysema is a rare cystic lesion of the lung which may be misdiagnosed and managed as pneumonia or pneumothorax. Case presentation: We presented a case of a congenital cystic lung malformation, the case demonstrated a 29-day-old boy who presented with respiratory distress. He was initially diagnosed as pneumonia and was given unnecessary antibiotic treatment in another hospital without improvement. Subsequently, the diagnosis of congenital lobar emphysema of the left upper lobe was made on the basis of the clinical and radiologic features. The condition wasn't detected before birth. He underwent a successful left thoracotomy with left upper lobe lobectomy. Clinical discussion: Congenital Lobar Emphysema poses a challenge in diagnosis. It may mimic other causes of respiratory distress. Conclusion: Congenital Lobar Emphysema requires a high index of clinical and radiological suspicion to make an early diagnosis so that timely treatment will be offered.

Ollier disease: the first report in Syria.

Ollier disease is a rare nonhereditary skeletal disorder, characterized by multiple enchondromas, which are noncancerous growth of cartilage. In this report, we present a case of Ollier disease in a 10-year-old Syrian boy. The patient presented with multiple boney masses on hands; he had a history of pathological fractures when he was 5, which caused crippling. We analyzed the clinical, radiographical and pathological characteristics of our patient, which helped us to reach the final diagnosis. Ollier disease is a benign bone tumor, but it has a risk of malignant transformation into chondrosarcoma. The aim of this report is to document the presence of Ollier disease in Syria to help other Syrian physicians considering this disease in the differential diagnosis if they face similar presentations.