Management of Acute Complications of Sickle Cell Disease.

Sickle cell disease (SCD) is a group of inherited autosomal recessive disorders that affect hemoglobin structure. The presence of this mutated form of hemoglobin, hemoglobin S, results in the abnormally ("sickle") shaped cells. These sickle-shaped red blood cells lead to the disruption of blood flow in small vessels and result in a myriad of complications. Pain, excruciating and unpredictable, is the hallmark of the disease. In addition, many organs are affected, including but not limited to brain, kidneys, bones, and lungs. This leads to varied acute and chronic complications for patients with SCD. Here, we review some of the acute complications of SCD with focus on diagnosis and management. [Pediatr Ann. 2024;53(2):e47-e51.].

Exudative Retinal Detachment Following Chimeric Antigen Receptor T-Cell Therapy in Relapsed B-Cell Acute Lymphoblastic Leukemia.

Chimeric antigen receptor (CAR) T-cell therapy has become a novel approach in the treatment of many hematologic malignancies. However, ocular adverse effects have not been well described. This report presents a case of a pediatric patient with relapsed B-cell acute lymphoblastic leukemia with ocular involvement treated with CAR T-cell therapy who developed an exudative retinal detachment likely secondary to an inflammatory response to CAR T-cell therapy. [Ophthalmic Surg Lasers Imaging Retina. 2022;53:113-115.].

Hemorrhagic Shock after Neonatal Circumcision: Severe Congenital Factor XIII Deficiency.

A Caucasian male infant born full term via normal spontaneous vaginal delivery was given vitamin K after birth, circumcised on day of life (DOL) 1, and discharged from the nursery on DOL 2. At the time of circumcision, oozing from the surgical site was noted and initially resolved with silver nitrate. Over the next two days, he presented to local emergency rooms multiple times for recurrent bleeding, eventually developing hemorrhagic shock resulting in admission to the neonatal intensive care unit. After extensive work up, he was ultimately diagnosed with severe congenital factor XIII deficiency. Congenital factor XIII deficiency is a rare bleeding disorder characterized by normal prothrombin time (PT) and activated partial thromboplastin time (aPTT) coagulation labs on routine screening, and has a high risk of complications, such as spontaneous intracranial hemorrhage. Although uncommon, when caring for a child with bleeding, physicians must have a high index of suspicion to make this diagnosis in order to initiate proper treatment and start prophylaxis given the risk of morbidity and mortality in untreated patients.