Successful treatment of dumping syndrome with diazoxide in an infant with hypoplastic left heart syndrome.

Summary: Dumping syndrome is a rare but potentially serious condition that causes inappropriate postprandial hyperinsulinemia leading to hypoglycemia in children following gastrointestinal surgeries. While dietary modifications are often the first line of treatment, severe cases may require pharmacological intervention to prevent severe hypoglycemia. We present a case of successful treatment of dumping syndrome with diazoxide. A 2-month-old infant with left hypoplastic heart syndrome who underwent single ventricle palliation pathway and developed feeding intolerance that required Nissen fundoplication. Postprandial hypoglycemia was detected following the procedure, with glucose level down to 12 mg/dL, and the diagnosis of dumping syndrome was established. The patient was successfully managed with diazoxide, which effectively resolved postprandial hypoglycemia without any major adverse events. The patient was eventfully weaned off the medication at the age of 5 months. This case highlights the potential role of diazoxide in the management of pediatric patients with postprandial hyperinsulinemic hypoglycemia secondary to dumping syndrome. Learning points: Dumping syndrome is a possible complication of gastrointestinal surgeries and should be suspected in children with abnormal glucose levels. Postprandial hyperglycemia should be monitored closely for significant subsequent hypoglycemia. Diazoxide might be considered as part of the treatment plan for dumping syndrome.

Infective intracardiac lesion in the setting of Mycobacterium franklinii bacteremia identified by cell-free DNA sequencing in a child with acute lymphoblastic leukemia: A potential new foe in intracardiac infections.

Mycobacterium franklinii (Mfra) is a recently identified member of the Mycobacterium chelonae-abscessus complex (MCAC), a rapidly growing, acid-fast bacilli that have the potential to cause invasive human infections. Identification of Mfra is crucial for selecting the appropriate antimicrobial therapy, as Mfra displays a unique susceptibility profile compared to other MCAC members. The literature on Mfra is limited, with a few studies focusing on respiratory and skin infections. To our knowledge, we describe the first reported case of cardiac involvement associated with Mfra bacteremia in a patient with acute lymphoblastic leukemia. The isolation of Mfra through a next-generation sequencing test allowed for prompt identification and subsequent implementation of tailored antimicrobial agents, ultimately resulting in positive clinical outcomes. This case also emphasizes the significance of next-generation testing in managing immunocompromised patients with persistent fever.

Endocardial fibroelastosis in infants and young children: a state-of-the-art review.

Endocardial fibroelastosis (EFE) is a rare cardiac condition characterized by excessive endocardial thickening secondary to fibroelastic tissues that commonly present in infants and young children. Most of endocardial fibroelastosis cases are secondary forms, which occur in conjunction with other cardiac diseases. Endocardial fibroelastosis has been associated with poor prognosis and outcomes. In light of recent advancements in understanding pathophysiology, several new data have revealed compelling evidence that abnormal endothelial-to-mesenchymal transition is the root cause of endocardial fibroelastosis. This article aims to review the recent development in pathophysiology, diagnostic workup, and management, and to discuss possible differential diagnoses.

Isolated left subclavian artery with right aortic arch: case report and literature review of 50 cases.

Isolated left subclavian artery (ILSA) with right aortic arch is a rare vascular anomaly accounting for 0.8% in all right-sided aortic arch anomalies. We report a case of an isolated left subclavian artery with right aortic arch and a combination of subclavian as well as pulmonary steal in infant with Tetralogy of Fallot. We reviewed and summarized 50 similar cases reported in the literature over the last 30 years, in order to gain a thorough understanding of this rare anomaly.

Role of Particulated Juvenile Cartilage Allograft Transplantation in Osteochondral Lesions of the Talus: A systematic review.

INTRODUCTION/PURPOSE: Osteochondral lesion of the talus (OCLT) is defined as a defect on the articular surface of the talus with/without subchondral bone involvement. Several surgical techniques are described in literature to treat OCLT. Particulated Juvenile Cartilage Allograft Transplantation (PJCAT) is a new emerging technique that has the potential of restoring the native cartilage. However, available data on PJCAT in treating OCLT is confined to a small number of studies with heterogeneous population. The aim of this study is to systematically review the literature on clinical and radiological outcomes of PJCAT in treating OCLT. METHODS: A systematic search of Medline database was performed using Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. Number of patients and demographic data included age, gender and body mass index (BMI) were extracted. The level of evidence of each included study was identified. When feasible, mechanism of injury, lesion size and average follow up were recorded. The American Orthopedic Foot and Ankle Society Score (AOFAS), and Foot and Ankle Outcome Score (FAOS) were obtained to assess the functional outcomes. Magnetic Resonance Observation of Cartilage Repair Tissue (MOCART) score was selected to evaluate the radiological outcomes. RESULTS: A total of 10 studies were eligible for this review involving 132 patients. Postoperative AOFAS scores were available for 44 patients who underwent PJCAT with an average of 86.14 at 25.5 months follow up. FAOS scores have been reported in 3 studies for 81 patients. The average preoperative score was 47.35 which has been improved to 62.88 at follow up of 23.6 months. MOCART was evaluated in 42 feet after the mean follow up of 18.2 months. The repair tissue was characterized by variable features; however, certain peculiarities were observed with higher frequency including infill hypertrophy, incomplete border zone integration, deep surface disruption, structure inhomogeneity, patches of hyperintense signals, damage of subchondral lamina and subchondral bone, adhesions and absence of effusion. CONCLUSION: PJCAT seems to be a promising modality of treatment for OLT in terms of functional outcomes. Fair attempts of defect filling can be confirmed with MRI. Nevertheless, heterogeneous picture of regenerate cartilaginous tissue and lack of repair in subchondral bone and subchondral lamina are not in favor with claims of full restoration of lost normal hyaline articular cartilage.

The Association Between Social Jetlag and Glycemic Control in Diabetic Patients at King Saud University Medical City.

Social jetlag (SJL) has been linked to many cardiovascular and metabolic diseases, as it disturbs the circadian rhythm. In this study, we analyzed the impact of SJL on glycemic control. To our knowledge, this was the first study that discussed the issue of SJL, and we explored the prevalence of SJL in the studied population. A case-control study matched by age and gender was conducted among 511 subjects. Control group subjects were diabetic with HbA1c levels of <7.5%, while our cases were diabetic with HbA1c levels of 7.5% or more. We used the Munich Chronotype Questionnaire (MCTQ) to assess SJL among the participants. Based on our findings, SJL status was similar for both cases and control participants, which indicates that there is no significant association between SJL and HbA1c levels (p=0.394). The prevalence of SJL in the studied population was 58.4%. Further studies are required to obtain a more precise estimation of sleep duration and SJL, and they should focus on SJL and its related problems.

Top 100 Cited Articles on Lumbar Spondylolisthesis: A Bibliographic Analysis.

STUDY DESIGN: Bibliographic analysis. OBJECTIVE: The aim of this study is to identify the most cited studies on lumbar spondylolisthesis and report their impact in spine field. METHODS: Thomson Reuters Web of Science-Science Citation Index Expanded was searched using title-specific search "spondylolisthesis." All studies published in English language between 1900 and 2019 were included with no restrictions. The top 100 cited articles were identified using "Times cited" arranging articles from high to low according to citation count. Further analysis was made to obtain the following items: article title, author's name and specialty, country of origin, institution, journal of publication, year of publication, citations number, study design. RESULTS: The citation count of the top 100 articles ranged from 68 to 589. All published between 1932 and 2016. Among 20 journals, Spine had the highest number of articles (49), with citation number of 6155 out of 13 618. Second ranked was Journal of Bone and Joint Surgery with 15 articles and total citations of 3023. With regard to the primary author's specialty, orthopedic surgeons contributed to the majority of top 100 list with 82 articles, and neurosurgery was the second specialty with 11 articles. The United States had produced more than half of the list with 59 articles. England was the second country with 7 articles. Surgical management of degenerative lumbar spondylolisthesis was the most common discussed topic. CONCLUSION: This article identifies the top 100 influential articles on lumbar spondylolisthesis and recognizes an important aspect of knowledge evolution served by leading researchers as they guided today's clinical decision making in spondylolisthesis.