Tetralogy of Fallot With Absent Left Pulmonary Artery in a Patient Who Passed Critical Congenital Heart Disease Screening.

The literature on tetralogy of Fallot (TOF) is abundant. It is a fairly common cyanotic congenital heart disease. It results from the anterior malalignment of the conal septum resulting in the aberrant formation of the ventricular septum leading to a defect. This presents a very important clinical significance because the prognosis usually depends on the clinical evaluation and initiation of timely therapy. We present a two-week-old baby with normal birth history and an uncomplicated newborn nursery course. He also passed the Critical Congenital Heart Disease screen at 24 hours of life. The routine examination by the pediatrician led to further investigations and treatments, highlighting the importance of good history-taking and clinical examination skills.

The Ominous Triad of Severe Takotsubo Cardiomyopathy.

QT prolongation is present in 26-52% of cases of Takotsubo cardiomyopathy (TCM). It has been postulated to result from reduced cardiac repolarization reserve and reflects the transient myocardial insult observed in TCM. Bradycardia-induced QT interval prolongation is amplified by the occurrence of TCM, a combination that potentially carries a significant risk for torsade de pointes (TdP). We present a unique case of an 80-year-old female with TCM-related cardiac arrest. The patient had acquired long QT syndrome in which TCM myocardial insult led to the precipitation of a third-degree atrioventricular (AV) block and subsequent bradycardia-induced TdP. Due to the lack of robust literature, there is no clear guideline in the management of third-degree AV block in the setting of TCM. In our case, because of recurrent ventricular tachycardia (VT) and ventricular fibrillation (VF) arrest, we opted for temporary pacing at a high ventricular rate, followed by a biventricular implantable cardioverter-defibrillator (BiV/ICD). Follow-up 3 months later revealed improvement of left ventricular (LV) dysfunction and resolution of QT prolongation. However, the noticed AV conduction defects persisted. In the available literature, we identified five reported cases that bear similarity with our patient's presentation. The identified cases were middle-aged to elderly females with no significant cardiac history, who exhibited a similar triad of TCM associated with high-grade AV block, acquired long QT syndrome, and a rapid progression of bradycardia-induced TdP, resulting in a near cardiac arrest within the first 24 - 48 h of admission. It is crucial to monitor corrected QT (QTc), correct electrolyte abnormalities, and minimize QT-prolonging medications in patients with TCM. The recognition of AV conduction defects in patients with TCM is critical, especially if it is associated with significant QT prolongation. Such situations are underrecognized, and are potentially fatal, necessitating close monitoring and timely intervention.

Accidental Ingestion of Tetrahydrocannabinol-Laced Gummies Causing Bradycardia and First-Degree Atrioventricular Block in a Pediatric Patient: A Case Report.

An increasing trend of cannabis use places children at risk for the detrimental effects of marijuana. Poison control centers in the United States have been experiencing an upsurge in calls involving marijuana ingestion among children in the past years, specifically in states where marijuana is legal. With marijuana ingestion, neurologic symptoms predominate but cardiovascular manifestations have also been observed. Bradycardia and bradyarrhythmia are both uncommon cardiac manifestations of cannabis ingestion in children. Here, we present the case of a previously healthy two-year-old male with sinus bradycardia and first-degree atrioventricular (AV) block following accidental ingestion of tetrahydrocannabinol-laced gummies. Although bradycardia and first-degree AV block are uncommon after cannabis ingestion in children, clinicians should be aware of these findings and must consider evaluating for marijuana toxicity whenever presented with these acute signs. Prevention is crucial and can be achieved through supervision, parental education, and support.

Daptomycin-Associated Diarrhea: A Case Report and Review of the Literature.

Antibiotic-associated diarrhea (AAD) describes any unexplained diarrhea associated with the use of antibiotics. AAD develops through diverse mechanisms, ranging from pharmacologic effects on gut motility to disturbance of the function and carbohydrate metabolism of the indigenous intestinal flora and overgrowth by pathogenic micro-organisms. Clostridioides difficile-associated diarrhea (CDAD) is a subset of AAD; however, it accounts only for a small percentage of diarrhea caused by antibiotics. Diarrhea has been reported as a side effect of daptomycin use, nevertheless, it's thought to be mild and carries significantly less risk of diarrhea than other alternative treatments of S. aureus bacteremia, i.e., vancomycin or cefazolin. The authors present an interesting case of daptomycin-associated diarrhea presenting with a protracted and severe course. Patient symptoms didn't improve with empiric Clostridioides difficile therapy and CDAD testing was negative. Diarrhea promptly resolved after discontinuation of daptomycin. Furthermore, a thorough literature review was conducted and discussed in this article to raise awareness of this under-recognized complication. Clinicians should be mindful of daptomycin-associated diarrhea along with its presentation and treatment. Further studies are needed to identify the pathophysiology of daptomycin-associated diarrhea and other forms of AAD. Understanding their mechanism could help prevent, treat, and reduce the significant medical costs associated with antibiotic adverse events.

New Phenotypic Feature in a Patient With a Rare Triplication of the 22q11.2 Region Presenting With Peters Anomaly, Congenital Heart Disease, and Global Developmental Delay: A Case Report and Literature Review.

The vulnerability of chromosome 22q11.2 region to rearrangement is due to several low copy repeat (LCR) sequences. These rearrangements are involved in syndromes that share similar phenotypic features. The rearrangements of the 22q11.2 chromosomal region are common, specifically, duplications and deletions associated with congenital anomalies and developmental disabilities disorders. However, the features associated with this chromosomal rearrangement remain largely unknown. We present, to the best of our knowledge, the third patient affected by triplication of the 22q11.2 chromosome region, who presents with Peters anomaly, global developmental delay, patent ductus arteriosus, and subaortic stenosis. This case highlights a new phenotypic feature associated with triplication of this genomic region.

Primary Psoas Abscess in a Pediatric Patient: A Case Report.

A psoas abscess is described as a collection of pus in the iliopsoas muscle compartment, which comprises the psoas and iliacus muscles located in the extraperitoneal space. It can be considered a primary abscess due to hematogenous or lymphatic seeding from a distant site, primarily occurring in children in tropical or developing countries. These primary infections are typically due to a single microorganism, most commonly, Staphylococcus aureus. Secondary spread develops due to the direct spread of infection of the psoas muscle from an adjacent structure (hip, vertebrae, gastrointestinal tract, aorta, genitourinary tract), developing due to trauma or instrumentation of the inguinal region, lumbar spine, or hip region. The secondary infections can be either mono- or polymicrobial and include enteric and anaerobic organisms. We present a case of psoas abscess in a five-year-old female who presented with a progressively worsening pain in the right hip for three days with refusal to bear weight and no history of trauma. Hip x-ray imaging yielded no abnormal results, but laboratory values prompted further investigation, leading to identifying a right psoas abscess via MRI with surrounding edema and enhancement. Imaging modality choice has proven to be instrumental in identifying psoas abscess and is key to achieving a diagnosis.

Thoracic Spinal Cord Compression Secondary to Metastatic Papillary Thyroid Carcinoma: An Unusual Oncological Phenomenon.

Hematogenous spread is fairly an unusual feature for papillary thyroid carcinoma (PTC) in comparison to follicular thyroid carcinoma (FTC). Thoracic spinal metastasis with complicating cord compression is an even rarer manifestation of PTC that was reported in a limited number of cases in the literature. Herein we present a 65-year-old female with a history of PTC on current radiotherapy, status post attempted surgery due to significant tumor burden and intraoperative bleeding, presented with a one-week history of rapidly progressive bilateral lower extremities weakness. Physical examination revealed paraplegia of both lower extremities with areflexia and a sensory level equivalent to the upper thoracic vertebrae. Urgent imaging depicted destructive epidural lesions at T1-T3 vertebrae with thoracic cord compression. Emergent laminectomy and debulking of these lesions were undertaken. Histopathological examination confirmed metastatic PTC. The patient proceeded to further treatment with radiotherapy following her successful neurological recovery. Thoracic vertebral metastasis is an unusual oncological phenomenon of PTC. Metastatic PTC should be considered in patients with a current or remote history of PTC who present with thoracic cord compression. Our case demonstrates that multidisciplinary management is the key to achieving a better outcome for metastatic PTC with thoracic cord compression.

Lutetium-177 Dotatate-Induced Hemolytic Anemia and Myelodysplastic Syndrome.

Lutetium-177 (177Lu) dotatate is a type of peptide receptor radioligand therapy (PRRT) using radiolabeled somatostatin for patients with progressive somatostatin receptor-positive gastrointestinal neuroendocrine tumors. While cases of therapy-related myeloid neoplasms (t-MN) have been described as a consequence of 177 Lu dotatate, there are no reports of hemolytic anemia associated with therapy. We present a case of a 68-year-old woman with metastatic low-grade neuroendocrine tumor who presented four weeks after the second dose of 177Lu dotatate with progressive fatigue and dyspnea. Laboratory workup was remarkable for hemolytic anemia. Lutetium-177 dotatate-induced hemolysis was suspected after ruling out other causes. Corticosteroid treatment was initiated with improvement in hemoglobin, and dose-reduced PRRT was planned upon discharge. Six months into the treatment course of 177Lu dotatate, macrocytic anemia was noticed on routine follow-up with normal vitamin B12 and folic acid levels. A bone marrow biopsy was done, revealing myelodysplastic syndrome (MDS) features. Given the temporal relationship between drug introduction and the objective findings, early-onset 177Lu dotatate-induced MDS was diagnosed with a plan for close hematologic follow-up. Myelodysplastic syndrome should be suspected when megaloblastic anemia develops in patients with previous 177Lu dotatate therapy. The latency period between initial treatment and MDS diagnosis reported in the literature ranges between 15 months to seven years. Apart from the unusually early onset of MDS, what is unique about our case is the development of hemolytic anemia after administration of PRRT. The clinical course and the brisk response to steroid therapy, suggest other mechanisms of PRRT toxicity besides DNA breaks, genetic mutations, and myelosuppression by an immune-mediated component that likely plays a role in 177Lu dotatate toxicity. Further investigation and monitoring are needed to identify the frequency of such adverse events and the pathophysiology of their occurrence.

Incomplete Kawasaki Disease in an Infant: A Case Report and Literature Review.

The term "incomplete Kawasaki Disease (IKD)" was first used to describe patients with coronary complications who did not fulfill the classical diagnostic criteria for Kawasaki Disease (KD). The risk of coronary artery involvement is similar if not greater in cases of IKD. However, the recognition of IKD is challenging and often delayed, especially in infants. Multiple algorithms have been formulated to identify cases of IKD utilizing supplemental clinical, echocardiographic, and laboratory features. Although fever is not required for a diagnosis of KD in the Japanese guideline, most of the current guidelines, including those of the American Heart Association (AHA), consider the presence of fever for at least seven days a requirement for the diagnosis of both KD and IKD in infants. We present a case of IKD in a four-month-old female who presented with fever for less than three days and did not follow the current AHA algorithm for IKD. An echocardiogram obtained 10 days later revealed a coronary artery aneurysm, and a retrospective diagnosis of IKD was made. A review of the literature identified similar cases with a growing consensus on the need to redefine the role of fever. Pediatricians should search for coronary artery lesions in cases of high clinical suspicion, even if the fever period is short, particularly in those less than six months. Additionally, further innovative research is directly needed to identify immunological and cellular markers that could be tested early in the course of the disease and guide the management.

The Diagnostic Dilemma of Acute Granulomatous Hepatitis in a Patient With Crohn's Disease: A Case Report and Review of Literature.

Liver involvement is not an uncommon extraintestinal manifestation of inflammatory bowel disease (IBD). IBD-associated liver diseases may have a variety of etiopathogenetic origins (including shared autoimmune pathogenesis, the effect of chronic inflammatory status, and adverse effects of drugs). Nevertheless, acute granulomatous hepatitis in the setting of Crohn's disease (CD) is a rare clinical entity. It warrants, however, a careful assessment as both clinical and pathological features of Crohn's-associated granulomatous hepatitis closely mimic extrapulmonary hepatic sarcoidosis, with considerable overlaps between the 2 diseases, which certainly makes a definitive diagnosis quite challenging. It is crucial to exclude infectious etiologies during the evaluation of acute granulomatous hepatitis, as inappropriate immunosuppressive treatment may cause a systemic flare-up of an underlying liver infection. We report a rare case of a 35-year-old female with a history of CD who presented with recurrent fevers, acute abdominal pain, and cholestasis. She was found to have acute hepatitis with noncaseating granulomas on liver biopsy. A comprehensive diagnostic workup did not ultimately prove a specific etiological culprit. The patient was treated with oral corticosteroids, and she demonstrated a positive clinical and laboratory response to the treatment. Our case highlights the diagnostic dilemma of acute granulomatous hepatitis in the setting of co-existent CD with a multisystemic syndrome. Granulomatous hepatitis represents a relatively rare manifestation of both extraintestinal CD and extrapulmonary sarcoidosis, with potential difficulties discriminating between the 2 entities on many occasions. The case also demonstrates the value of an interdisciplinary approach in the context of multisystemic disease to achieve the best outcome.

Early Hyporegenerative Anemia Complicating Hemolytic Disease of the Newborn Secondary to Rhesus Alloimmunization.

Rhesus hemolytic disease of the newborn is rarely found after the implementation of anti-D immunoglobulin prophylaxis. However, it may lead to cholestasis, elevated liver transaminases, hyperbilirubinemia, kernicterus, iron overload, and hyporegenerative anemia. Hyporegenerative anemia is characterized by low hemoglobin and reticulocyte count. It is typically recognized two to six weeks after birth. The etiology of this type of anemia is not identified yet, and treatment is controversial. We report a case of a neonate with rhesus hemolytic disease of the newborn with early hyporegenerative anemia that was noted on day seven of life. The available literature has described a similar age of onset, but after two weeks of life and not as early as on day seven of life as in our case. We treated this type of anemia with the standard of care management that includes phototherapy, intravenous immunoglobulin, and blood transfusions.