Prenatal delta-9-tetrahydrocannabinol exposure alters fetal neurodevelopment in rhesus macaques.

Prenatal cannabis use is associated with adverse offspring neurodevelopmental outcomes, however the underlying mechanisms are relatively unknown. We sought to determine the impact of chronic delta-9-tetrahydrocannabinol (THC) exposure on fetal neurodevelopment in a rhesus macaque model using advanced imaging combined with molecular and tissue studies. Animals were divided into two groups, control (n = 5) and THC-exposed (n = 5), which received a daily THC edible pre-conception and throughout pregnancy. Fetal T2-weighted MRI was performed at gestational days 85 (G85), G110, G135 and G155 to assess volumetric brain development. At G155, animals underwent cesarean delivery with collection of fetal cerebrospinal fluid (CSF) for microRNA (miRNA) studies and fetal tissue for histologic analysis. THC exposure was associated with significant age by sex interactions in brain growth, and differences in fetal brain histology suggestive of brain dysregulation. Two extracellular vesicle associated-miRNAs were identified in THC-exposed fetal CSF; pathway analysis suggests that these miRNAs are associated with dysregulated axonal guidance and netrin signaling. This data is indicative of subtle molecular changes consistent with the observed histological data, suggesting a potential role for fetal miRNA regulation by THC. Further studies are needed to determine whether these adverse findings correlate with long-term offspring neurodevelopmental health.

Resolution of Anastomotic Ulceration and Profound Bleeding by Endoscopic Cauterization and Oral Budesonide.

Anastomotic ulceration is a known complication of short gut syndrome, which can be complicated by concurrent iron deficiency and chronic bleeding. Diagnosis is confirmed through endoscopy, typically performed for the investigation of severe anemia. Inflammatory colitis in short gut syndrome has been previously reported; therefore, it is important to treat both ulceration and inflammation. Currently, no effective therapies are available. Herein, we describe the case of a child with short gut syndrome who subsequently developed anastomotic ulcers with recurrent severe bleeding and was successfully treated with endoscopic argon plasma cauterization for circumferential ulcerations, followed by a prolonged course of oral enteric budesonide. This intervention could be a potential and beneficial nonsurgical treatment for anastomotic ulceration.

A rare case of primary gastric Hodgkin lymphoma in an adolescent with Nijmegen breakage syndrome.

BACKGROUND: Nijmegen Breakage Syndrome (NBS) is a rare autosomal recessive DNA repair disorder that increases risk of hematological malignancy. Primary gastric malignancies are exceedingly rare in pediatric patients and not typically high on the differential of abdominal pain. CASE PRESENTATION: A 14-year-old male with NBS presented with persistent abdominal pain and was diagnosed with primary Hodgkin disease of the stomach. CONCLUSIONS: In pediatric patients with predisposition to malignancies, such as those with underlying chromosome instability disorders, all symptoms must be carefully considered.

A review of histopathologic features of pediatric autoimmune liver disease.

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Operative Technique: Angiomatoid Fibrous Histiocytoma-Unique Case and Management.

Objective We describe the first jugular foramen angiomatoid fibrous histiocytoma (AFH) case and the first treatment with preoperative endovascular embolization. AFH is a rare intracranial neoplasm, primarily found in pediatric patient extremities. With an increase in AFH awareness and a well-described genetic profile, intracranial prevalence has also subsequently increased. Study Design We compare this case to previously reported cases using PubMed/Medline literature search, which was performed using the algorithm ["intracranial" AND "angiomatoid fibrous histiocytoma"] through December 2020 (23 manuscripts with 46 unique cases). Patient An 8-year-old female presented with failure to thrive and right-sided hearing loss. Work-up revealed an absence of right-sided serviceable hearing and a large jugular foramen mass. Angiogram revealed primary arterial supply from the posterior branch of the ascending pharyngeal artery, which was preoperatively embolized. Intervention Gross total resection was performed via a translabyrinthine approach. Conclusion The case presented is unique; the first reported AFH at the jugular foramen and the first reported case utilizing preoperative embolization. Preoperative embolization is a relatively safe technique that can improve the surgeon's ability to perform a maximally safe resection, which may decrease the need for adjuvant radiation in rare skull base tumors in young patients.

Pediatric cystectomy for refractory cystitis post-bone marrow transplant in dyskeratosis congenita: A case report.

We present a case of refractory cystitis after BK-virus associated hemorrhagic cystitis following bone marrow transplantation requiring cystectomy in a 17-year-old female with genetically confirmed dyskeratosis congenita, a telomere disorder characterized by early bone marrow failure. She presented with a contracted, small bladder with intense urinary symptoms non-responsive to conservative therapy and requiring opioids for pain control. Cystectomy is a rare, final surgical treatment for benign bladder conditions, especially among younger patients, and she experienced successful resolution of symptoms and cessation of chronic opioids post-intervention.

A left atrial hamartoma of mature cardiac myocytes.

We present a case of a hamartoma of mature cardiac myocytes. This is an extremely rare tumour and the first reported paediatric case localised in the left atrium.

Updates in Pediatric Glioma Pathology.

Gliomas are a diverse group of primary central nervous system tumors with astrocytic, oligodendroglial, and/or ependymal features and are an important cause of morbidity/mortality in pediatric patients. Glioma classification relies on integrating tumor histology with key molecular alterations. This approach can help establish a diagnosis, guide treatment, and determine prognosis. New categories of pediatric glioma have been recognized in recent years, due to increasing application of molecular profiling in brain tumors. The aim of this review is to alert pediatric pathologists to emerging diagnostic concepts in pediatric glioma neuropathology, emphasizing the incorporation of molecular features into diagnostic practice.

Lymphocytic Colitis With Increased Apoptosis: A Marker of Mutation in T-Cell-Mediated Immunity?

Lymphocytic colitis is a subtype of microscopic colitis that is mostly seen in adults. It presents mainly as chronic nonbloody diarrhea, with the hallmark of normal or near-normal endoscopy. In this case series, we are presenting 4 pediatric patients with lymphocytic colitis with prominent apoptosis of the colonic gland epithelium. Remarkably, all the patients have genetic mutations known to be associated with autoimmune enteropathy. Three patients have a CTLA4 mutation, and 1 patient has an STAT3 mutation. These mutations were previously reported in association with inflammatory bowel disease, but a specific connection with lymphocytic colitis has not been described. This report investigates the histopathology of such lesions in children and adolescents.

Neuroendocrine carcinoma of the pineal parenchyma. The first reported case.

We report a case of a 35-year-old female patient who presented with worsening headaches, vertigo, and vision changes. MRI of the brain showed an enhancing lesion in the pineal region. The patient was taken for resection of the lesion which was classified as neuroendocrine carcinoma of the pineal parenchyma, intermediate grade. Histologically, the neoplasm was cellular, mitotically active, and composed of tightly packed cells with high nuclear cytoplasmic ratio, scant cytoplasm, and ill-defined cell borders. Immunohistochemically the tumor cells were positive for chromogranin, synaptophysin and AE1/AE3, and negative for CK-7, CK-20, and TTF1. Possible metastasis from any other primary sites was ruled out clinically. This represents the first reported case of neuroendocrine carcinoma of the pineal parenchyma.