Acute chest syndrome in pediatric sickle cell disease: A 19-year tertiary center experience.

INTRODUCTION: The most common cause of death among sickle cell disease (SCD) patients is acute chest syndrome (ACS). Since SCD is a common condition in the Eastern province of the Kingdom of Saudi Arabia (KSA), we aimed to provide a detailed description of the clinical characteristics and ACS management. METHODS: We retrospectively studied pediatric (<14 years) patients with SCD diagnosis who were admitted with ACS or developed ACS after admission from January 2002 to December 2020. The absence of chest X-ray or hemoglobin electrophoresis was the reason to exclude patients from the study. The primary objective of the study was to evaluate and report the clinical, laboratory, and management characteristics of ACS. RESULTS: Ninety-one ACS episodes (42 patients) were included, with a mean diagnosis age of 7.18 ± 3.38 years. Twenty-two (52.4%) patients were male. Twenty-five patients had recurrent ACS episodes. The median absolute number of ACS was 3.5 (interquartile range [IQR], 2-9), with maximum ACS episodes of 13/1 year and a minimum of 1 ACS episode per year. At the first ACS episode, the mean age was 6.62 ± 3.38 years, while the overall mean age at ACS episode diagnosis was 7.18 ± 3.38 years. The most common antecedent events were vaso-occlusive crisis (12 episodes, 13.2%) and upper respiratory tract infections (8 episodes, 8.8%). The most frequently encountered presenting symptoms were fever (70.3%) and cough (70.3%). The most common antibiotics used were azithromycin (82.4%) and ceftriaxone (75.8%). Nine patients (9.9%) required pediatric intensive care unit (PICU) admission. Of the 91 ACS episodes, there was no in-hospital mortality. The median hospital and PICU length of stay were 8 days (IQR, 5-10.25) and 4 days (IQR, 3-5.5), respectively. CONCLUSION: This study has reported the most common clinical characteristics and management of ACS among pediatric SCD patients in the Eastern province of KSA.

Predictors of Recurrent Acute Chest Syndrome in Pediatric Sickle Cell Disease: A Retrospective Case-Control Study.

Acute chest syndrome (ACS) is a common cause of death in sickle cell disease (SCD) patients. Multiple studies investigated the risk factors of developing ACS; however, predictors of recurrent ACS episodes have not been thoroughly investigated. We aim to examine the clinical and laboratory predictors of recurrent ACS in pediatric patients with SCD. A retrospective case-control study included pediatric patients with SCD (˂14 years) admitted with ACS or developed ACS during admission for another indication. Patients were classified into recurrent ACS episodes (≥2 episodes) and a single ACS episode groups. Ninety-one ACS episodes (42 patients) were included, with a mean age at diagnosis of 7.18 ± 3.38 years. Twenty-two (52.4%) patients were male, and twenty-five (59.5%) patients had recurrent ACS. Younger age at first ACS was significantly associated with recurrence (p = 0.003), with an optimal cutoff at 7.5 years (area under the receiver operating characteristic curve [AUROC] = 0.833; p < 0.001). Higher SCD-related hospitalizations were significantly associated with recurrence (p = 0.038). Higher mean values of baseline white blood count (WBC) (p = 0.009), mean corpuscular volume (MCV) (p = 0.011), and reticulocyte (p = 0.036) were significantly associated with recurrence. Contrarily, lower baseline hematocrit values (p = 0.016) were significantly associated with recurrence. The ACS frequencies were significantly lower after hydroxyurea (p = 0.021). The odds of ACS recurrence increased with a positive C-reactive protein (CRP) at admission (p = 0.006). In conclusion, several baseline and admission laboratory data showed significant associations with recurrence. Hydroxyurea therapy demonstrated reduced ACS episodes.

Adenomyoepithelioma of the Breast: A Report of 3 Cases.

BACKGROUND Breast adenomyoepithelioma is a rare benign breast tumor characterized by a biphasic proliferation of epithelial and myoepithelial cells with variable clinical and diagnostic features. Establishing the diagnosis, determining optimal therapy, and predicting outcome are problematic because of the rarity of this entity. There have been only 2 large series of adenomyoepitheliomas of the breast, reported by Tavassoli and Rosen, which included 27 and 18 patients, respectively. In this report, we present 3 cases of breast adenomyoepithelioma. CASE REPORT Herein, we report 3 cases of breast adenomyoepithelioma. The first case is of a 64-year-old woman who was found to have right breast microcalcification on a screening mammogram. The second case is of a 74-year-old woman who had a right breast mass. These 2 patients were managed by wide local excision. Postoperative microscopic examination revealed adenomyoepithelioma. The third case is of a 49-year-old woman with bilateral saline breast implants who presented with a left breast mass. A core needle biopsy was done and revealed adenomyoepithelioma associated with usual ductal hyperplasia and ductal carcinoma in situ. CONCLUSIONS Breast adenomyoepithelioma is a rare condition that can pose diagnostic challenges due to variable imaging presentations, necessitating percutaneous core biopsy for initial diagnosis. Correct diagnosis is usually possible only on excisional biopsy and confirmed by demonstrating the biphasic nature of the tumor by IHC. Clinical suspicion coupled with utilizing both radiological and histopathological facilities can aid in the accurate diagnosis and management. For the most part, they are considered to be benign, but they can locally recur.

Giant Esophageal Leiomyoma: Diagnostic and Therapeutic Challenges.

BACKGROUND Leiomyoma is a rare, benign, esophageal tumor that does not often measure >10 cm. Here, we report a case of giant esophageal leiomyoma in a 24-year-old man. CASE REPORT A 24-year-old man who smoked and had primary hypertension and glucose-6-phosphate dehydrogenase deficiency presented with a history of shortness of breath and productive cough with yellowish sputum, a long history of dysphagia to solid food, and a weight loss of 7 kg over 2 months. A chest X-ray revealed a mediastinum with a width >8 cm. Computed tomography of the patient's chest revealed a multilobulated mass that originated from the upper and middle thoracic esophagus, caused severe narrowing of his esophageal lumen, and was compressing his trachea and right main bronchus. Resection of the tumor was performed and, because of the large defect after the surgery and the mucosal necrosis, the patient underwent an Ivor-Lewis esophagectomy. His postoperative course was uneventful. He had no symptoms when he was seen in the outpatient clinic for follow-up and fully recovered. CONCLUSIONS Giant esophageal leiomyoma (GEL) is a rare oncological entity that presents several diagnostic and therapeutic challenges because of the scarcity of information in the medical literature on surgical management. The descriptions of techniques for surgical resection of GEL do not include ways to effectively perform subsequent reconstruction. The aim of the present paper was to contribute to this scant information by reporting our experience with performing an Ivor-Lewis esophagectomy to manage a case of GEL.

High-Flow Nasal Cannula Treatment in Patients with COVID-19 Acute Hypoxemic Respiratory Failure: A Prospective Cohort Study.

BACKGROUND: Early use of high-flow nasal cannula (HFNC) decreases the need for endotracheal intubation (EI) in different respiratory failure causes. While HFNC is used in coronavirus disease 2019 (COVID-19)-related acute hypoxemic respiratory failure (AHRF) under weak recommendations, its efficacy remains to be investigated. OBJECTIVES: The primary objective was to examine HFNC efficacy in preventing EI among COVID-19 patients with AHRF. Secondary objectives were to determine predictors of HFNC success/failure, mortality rate, and length of hospital and intensive care unit (ICU) stay. PATIENTS AND METHODS: This is a prospective cohort study conducted at a single tertiary care centre in Saudi Arabia from April to August 2020. Adult patients admitted to the ICU with AHRF secondary to COVID-19 pneumonia and managed with HFNC were included. We excluded patients who were intubated or managed with non-invasive ventilation before HFNC. RESULTS: Forty-four patients received HFNC for a median duration of 3 days (interquartile range, 1-5 days). The mean age was 57 ± 14 years, and 86% were men. HFNC failure and EI occurred in 29 (66%) patients. Patients in whom HNFC treatment failed had a higher risk of death (52% versus 0%; P = 0.001). After adjusting for confounding factors, a high SOFA score and a low ROX index were significantly associated with HFNC failure (hazard ratio [HR], 1.42; 95% confidence interval [CI], 1.04-1.93; P = 0.025; and HR, 0.61; 95% CI, 0.42-0.88; P = 0.008, respectively). CONCLUSIONS: One-third of hypoxemic COVID-19 patients who received HFNC did not require intubation. High SOFA score and low ROX index were associated with HFNC failure.