Lacrimal Gland Adenoid Cystic Carcinoma with High Grade Transformation: A Case Report and Current Concepts in Multi Modality Management.

Lacrimal gland adenoid cystic carcinoma (AdCC) is associated with an aggressive clinical course and grave prognosis. A high grade transformation within adenoid cystic carcinoma of lacrimal gland is a rare condition which is even more locally aggressive with frequent neck and distant metastasis. We present a case of left lacrimal gland adenoid cystic carcinoma with high grade transformation to adenocarcinoma NOS type presenting with orbital pain and proptosis. After thorough evaluation for locoregional and distant spread of the disease, the patient underwent left orbital exenteration with orbitectomy and neck dissection with free flap reconstruction. Patient received adjuvant radiation therapy and is presently disease free for last 6 months. A multi-modality management protocol involving surgery, radiotherapy and chemotherapy has been proposed for management of lacrimal gland AdCC with high grade transformation. We report the 4th case in the literature of lacrimal gland adenoid cystic carcinoma with high grade transformation.

A Survey of Hospital Pharmacy Guidelines for the Administration of 3% Sodium Chloride in Children.

Three percent sodium chloride (3% NaCl) is the treatment of choice for symptomatic hyponatremia. A barrier to the use of 3% NaCl is the perceived risk of both local infusion reactions and neurologic complications from overcorrection. We examine whether children's hospital pharmacies have policies or practice guidelines for the administration of 3% NaCl and whether these pharmacies have restrictions on the administration of 3% NaCl in terms of rate, route, volume and setting. An Internet survey was distributed to the pharmacy directors of 43 children's hospitals participating in the Children's Hospital Association (CHA) network. The response rate was 65% (28/43). Ninety-three percent (26/28) of pharmacy directors reported a restriction for the administration of 3% NaCl, with 57% restricting its use through a peripheral vein or in a non-intensive care unit setting, 68% restricting the rate of administration and 54% restricting the volume of administration. Seventy-one percent (20/28) reported having written policy or practice guidelines. Only 32% of hospital pharmacies allowed 3% NaCl to be administered through a peripheral IV in a non-intensive care unit setting. The majority of children's hospital pharmacies have restrictions on the administration of 3% NaCl. These restrictions could prevent the timely administration of 3% NaCl in children with symptomatic hyponatremia.

Emerging role of cannabinoids and synthetic cannabinoid receptor 1/cannabinoid receptor 2 receptor agonists in cancer treatment and chemotherapy-associated cancer management.

Cannabis was extensively utilized for its medicinal properties till the 19th century. A steep decline in its medicinal usage was observed later due to its emergence as an illegal recreational drug. Advances in technology and scientific findings led to the discovery of delta-9-tetrahydrocannabinol (THC), the primary psychoactive compound of cannabis, that further led to the discovery of endogenous cannabinoids system consisting of G-protein-coupled receptors - cannabinoid receptor 1 and cannabinoid receptor 2 along with their ligands, mainly anandamide and 2-arachidonoylglycerol. Endocannabinoid (EC) is shown to be a modulator not only for physiological functions but also for the immune system, endocrine network, and central nervous system. Medicinal research and meta-data analysis over the last few decades have shown a significant potential for both THC and cannabidiol (CBD) to exert palliative effects. People suffering from many forms of advanced stages of cancers undergo chemotherapy-induced nausea and vomiting followed by severe and chronic neuropathic pain and weight loss. THC and CBD exhibit effective analgesic, anxiolytic, and appetite-stimulating effect on patients suffering from cancer. Drugs currently available in the market to treat such chemotherapy-induced cancer-related ailments are Sativex (GW Pharmaceutical), Dronabinol (Unimed Pharmaceuticals), and Nabilone (Valeant Pharmaceuticals). Apart from exerting palliative effects, THC also shows promising role in the treatment of cancer growth, neurodegenerative diseases (multiple sclerosis and Alzheimer's disease), and alcohol addiction and hence should be exploited for potential benefits. The current review discusses the nature and role of CB receptors, specific applications of cannabinoids, and major studies that have assessed the role of cannabinoids in cancer management.

New-Onset Nephrotic Syndrome in a Child Associated With COVID-19 Infection.

Introduction: The COVID-19 outbreak has become a worldwide public health emergency. The renal histopathological features of acute tubular necrosis or thrombotic microangiopathy have been previously reported in adults with severe COVID-19 infections. In children, the renal manifestations associated with COVID-19 disease are not widely reported. Here we describe a case report of a child with new-onset nephrotic syndrome associated with COVID-19 infection. Case Presentation: An 8-year-old boy with no previous significant medical history presented with bilateral eyelid and facial swelling soon after his parents were diagnosed with COVID-19 infection. He had diarrhea but no fever or shortness of breath. At 1 week after the onset of swelling, the boy tested positive for the COVID-19 virus. Based on clinical findings of significant proteinuria (urine protein and creatinine ratio of 11.4), hypoalbuminemia (serum albumin of 2 g/dl), and hypercholesterolemia (total cholesterol of 384 mg/dl), he was diagnosed with nephrotic syndrome. He responded well to standard-dose prednisone treatment for nephrotic syndrome. At 1 week after starting the prednisone treatment, he went into clinical remission. Lymphopenia continued to be present for 4 weeks after the onset of symptoms. There were no complications related to clot formation or secondary infections with this presentation. Conclusion: COVID-19 can be associated with new-onset nephrotic syndrome in children. The patient responded well to the standard-dose prednisone treatment that is typically used for new-onset nephrotic syndrome. Summary: We describe the unique presentation of COVID-19 in a child as new-onset nephrotic syndrome. We offer insight on the success of standard treatment of nephrotic syndrome with COVID-19.

A Case Report of Baclofen Toxicity in a Pediatric Patient With Normal Kidney Function Successfully Treated With Hemodialysis.

RATIONALE: Baclofen is a commonly prescribed medication used to decrease spasticity in children with cerebral palsy. Despite its widespread use, this medication has not demonstrated to be consistently effective in clinical studies. Baclofen is also associated with systemic adverse effects due to potent neuronal depression. The management of baclofen toxicity is mainly supportive; however, some studies have shown that hemodialysis may alleviate the symptoms of an overdose and shorten the recovery time. PRESENTING CONCERNS: In this case report, a 6-year-old boy with mild cerebral palsy, neuromyelitis optica, and normal kidney function was found unresponsive at home, with altered mental status, after ingesting 1300 mg of baclofen unobserved. The patient was intubated and mechanically ventilated because of significant neurologic depression with subsequent respiratory failure. DIAGNOSIS: The patient was diagnosed with baclofen-induced encephalopathy. An elevated serum baclofen level of 4.00 µg/mL (therapeutic range of 0.08-0.40 µg/mL) was observed 10 hours after he was found unresponsive. The patient's respiratory status deteriorated; he had high ventilatory requirements and remained comatose. INTERVENTION: With the worsening of his clinical condition in the intensive care unit, hemodialysis, administered via a high-efficiency high-flux dialyzer, was initiated approximately 18 hours after he was found unresponsive. The patient underwent 2 hemodialysis runs spaced 9 hours apart, with blood flow rates approaching 250 mL/min. OUTCOMES: Within 3 hours of the first hemodialysis treatment, the patient started to regain consciousness. He was extubated to room air 6 hours after the second hemodialysis treatment. NOVEL FINDINGS: Supportive management is the primary treatment of baclofen toxicity in a pediatric patient with normal kidney function. Hemodialysis may be considered in severe cases of baclofen toxicity and worsening clinical status, but further studies are needed to confirm this finding.

JUSTIFICATION: Le baclofène est un médicament couramment prescrit pour réduire la spasticité chez les enfants atteints de paralysie cérébrale. Malgré son usage répandu, l'efficacité du baclofène n'a pas été démontrée de façon constante dans les études cliniques. Ce médicament est également associé à des effets secondaires systémiques en raison d'une forte dépression neuronale. La gestion de la toxicité du baclofène consiste principalement en un traitement de soutien. Certaines études ont toutefois démontré que l'hémodialyse pourrait atténuer les symptômes d'une surdose et hâter la convalescence. PRÉSENTATION DU CAS: Nous présentons les cas d'un garçon de six ans atteint d'une légère paralysie cérébrale et d'une neuromyélite optique. L'enfant, dont la fonction rénale était normale, avait été retrouvé inconscient à son domicile, avec un état mental altéré, après avoir ingéré accidentellement 1 300 mg de baclofène. L'importante dépression neurologique et l'insuffisance respiratoire subséquente ont nécessité l'intubation et la ventilation mécanique du patient. DIAGNOSTIC: Une encéphalopathie induite par le baclofène a été diagnostiquée. Un taux très élevé de baclofène sérique, soit de 4,00 µg/mL (l'intervalle thérapeutique se situe entre 0,08 et 0,40 µg/mL), a été observé dix heures après que l'enfant ait été retrouvé inconscient. Le patient demeurait comateux, son état respiratoire s'était dégradé et ses besoins ventilatoires étaient très élevés. INTERVENTIONS: À l'unité des soins intensifs, la dégradation de l'état clinique a nécessité l'amorce d'un traitement d'hémodialyse environ 18 heures après que l'enfant ait été retrouvé inconscient. Le traitement a été administré à l'aide d'un filtre avec une membrane à haute efficacité et à haute perméabilité. Le patient a subi deux séances d'hémodialyse à neuf heures d'intervalle, avec un débit sanguin approchant les 250 mL/min. RÉSULTATS: L'enfant a lentement repris conscience moins de trois heures après le début du premier traitement d'hémodialyse, et a été extubé six heures après le deuxième traitement d'hémodialyse. NOUVEAUX RÉSULTATS: Le principal traitement dans le cas d'une intoxication au baclofène chez un enfant avec une fonction rénale normale est la prise en charge symptomatique. L'hémodialyse pourrait être envisagée en cas d'intoxication grave avec dégradation de l'état clinique. D'autres études sont toutefois nécessaires pour confirmer ce résultat.

Renal transplantation in Bardet-Biedl Syndrome.

BACKGROUND: Renal anomalies are common in patients with Bardet-Biedl syndrome (BBS), a renal cystic ciliopathy with multi-systemic features. Renal transplantation is indicated in cases of end-stage renal disease (ESRD), but transplant centers may be hesitant to perform the necessary transplant in light of the multitude of metabolic comorbidities these patients often face with the potential to complicate outcomes. METHODS: Data from the Clinical Registry Investigating BBS (CRIBBS) were used to investigate renal transplant outcomes in the largest BBS cohort described to date. RESULTS: Of the 206 patients enrolled in the CRIBBS, 21 children (10.2 %; 16 girls, 5 boys; median age 8.4 years) had been diagnosed with ESRD. Renal transplantation was performed in 18 of these individuals between 1982 and 2015, including repeat transplantation in some cases, for a total of 22 kidneys. Overall graft survival was 81.6 % at 1 year post-transplantation, 75.7 % at 5 years, 59 % at 10 years, and 49.2 % at 25 years. Patient survival was 94.4 % at 1 year post-transplantation, 87.2 % at 8 years, and 79.3 % at 25 years. CONCLUSIONS: At a median follow-up time of 97 months, relatively few complications of renal transplantation were reported in the patients of this study. However, body mass index was significantly elevated in transplanted individuals compared to non-transplanted individuals participating in CRIBBS at the most recent follow-up. Although the frequency of obesity and other manifestations of the metabolic syndrome warrant meticulous management in this high-risk population, favorable long-term outcomes suggest that renal transplantation is a viable option for patients with BBS and ESRD.