Analysis of Ganciclovir-Resistant Cytomegalovirus Infection Caused by the UL97 Gene Mutation in Codons 460 and 520 in Pediatric Patients: A Case Series

Abstract Background Drug-resistant cytomegalovirus (CMV) infection has been increasingly recognized. However, there are limited data in pediatric patients. In this study, the prevalence and factors associated with CMV infection with UL97 mutations in pediatric patients treated with ganciclovir but not responding to treatment were evaluated. Methods This retrospective study was conducted from January 2013 to December 2017. All patients who were suspected of having ganciclovir-resistant CMV infection and had never had ganciclovir prophylaxis were included. Genotypic assay for UL97 mutations in codons 460 and 520 conferring ganciclovir resistance was performed. Factors associated with the presence of UL97 mutations were analyzed. Results Of 34 patients included, 10 patients (29.4%) had a genotypically confirmed UL97 mutation. The median age (interquartile range [IQR]) was 3 (0.85–8.68) years. Ganciclovir resistance was tested at a median time (IQR) of 22.5 (14.3–31) days after initiation of ganciclovir. All resistant isolates harbored a UL97 mutation in codon 460. Compared with patients infected with CMV without UL97 mutation, those infected with UL97 mutation strains were younger (median age [IQR], 3.02 [0.85–8.68] vs 10.45 [2.7–16.4] years) and had a higher maximum viral load (median [IQR], 5.06 [4.74–6.05] vs 4.42 [4.03–4.87] copies/mL). Six of 10 (60%) patients were successfully treated with high-dose ganciclovir (7.5 mg/kg twice daily). Conclusions UL97 mutation ganciclovir-resistant CMV infection was not uncommon in the pediatric population. Screening for this mutation should be considered in patients experiencing virological worsening while ganciclovir is given, even if patients have not previously received ganciclovir prophylaxis.

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Successful Use of Intravenous Methylnaltrexone for Opioid-Induced Constipation in Critically Ill Pediatric Patients

Journal of Pediatric Intensive Care ◽

10.1055/s-0041-1736335 ◽

2021 ◽

Author(s):

Kimberly P. Mills ◽

Christopher C. McPherson ◽

Ahmed S. Said ◽

Michael A. Lahart

Keyword(s):

Adverse Events ◽

Critically Ill ◽

Primary Outcome ◽

Pediatric Patients ◽

Case Series ◽

Opioid Withdrawal ◽

Limited Data ◽

Serious Adverse Events ◽

Patient Response ◽

Hospital Patients

Abstract Objectives Methylnaltrexone is U.S. Food and Drug Administration (FDA) approved as a subcutaneous injection for adults with opioid-induced constipation (OIC). Case series have described the use of methylnaltrexone for OIC in the pediatric oncology population. There are limited data describing its intravenous use in critically ill pediatric patients. Methods We conducted a retrospective observational study at St. Louis Children's Hospital. Patients less than 18 years old who received at least one dose of intravenous methylnaltrexone while admitted to an intensive care unit between January 2016 and August 2019 were included. The primary outcome was documented laxation within 24 hours of methylnaltrexone administration. Results Sixteen patients received a total of 34 doses of intravenous methylnaltrexone. Patients received a median of 1.69 (interquartile range [IQR], 0.9–4.86) morphine milligram equivalents per kilogram per 24 hours, over a median of 14 days (IQR, 11–30), before methylnaltrexone administration. The median dose of methylnaltrexone was 0.15 mg/kg (IQR, 0.15–0.16). Ten patients (63%) responded to the first dose of methylnaltrexone, and 14 patients (88%) responded to at least one dose. Overall, 26 doses (76%) led to patient response. Four patients (25%) experienced adverse events (emesis, abdominal pain) after methylnaltrexone administration. No signs or symptoms of opioid withdrawal were documented. Conclusions Intravenous methylnaltrexone appears to be safe and effective in treating OIC in critically ill pediatric patients. No serious adverse events or signs of opioid withdrawal were observed after single and repeat dosing. Patients responded to methylnaltrexone with varying opioid dosing and durations prior to administration.

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Robotic adrenalectomy in the pediatric population: initial experience case series from a tertiary center

BMC Urology ◽

10.1186/s12894-020-00727-x ◽

2020 ◽

Vol 20 (1) ◽

Author(s):

Anirban P. Mitra ◽

Evalynn Vasquez ◽

Paul Kokorowski ◽

Andy Y. Chang

Keyword(s):

Hospital Stay ◽

Pediatric Patients ◽

Pediatric Population ◽

Operating Time ◽

Case Series ◽

Length Of Hospital Stay ◽

Open Approach ◽

Invasive Approach ◽

Patients Âgés ◽

Tertiary Center

Abstract Background Laparoscopic resection is the most well described minimally-invasive approach for adrenalectomy. While it allows for improved cosmesis, faster recovery and decreased length of hospital stay compared with the open approach, instrument articulation limitations can hamper surgical dexterity in pediatric patients. Use of robotic assistance can greatly enhance operative field visualization and instrument control, and is in the early stages of adoption in academic centers for pediatric populations. Case presentation We present a single-institution series of pediatric adrenalectomy cases. The da Vinci Xi surgical system was used to perform adrenalectomies on three consecutive patients (ages, 2–13 years) at our center. Final pathology revealed ganglioneuroblastoma (n = 2) and pheochromocytoma (n = 1). Median operating time was 244 min (range, 244–265 min); median blood loss was estimated at 100 ml (range, 15–175 ml). Specimens were delivered intact and all margins were negative. Median post-operative hospital stay was 2 days (range, 1–6 days). All patients remain disease-free at median follow-up of 19 months (range, 12–30 months). Conclusion Our experience continues to evolve, and suggests that robotic surgery is safe, feasible and oncologically effective for resection of adrenal masses in well-selected pediatric patients.

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The transsphenoidal resection of pediatric craniopharyngiomas: a case series

Journal of Neurosurgery Pediatrics ◽

10.3171/2009.7.peds09252 ◽

2010 ◽

Vol 5 (1) ◽

pp. 49-60 ◽

Cited By ~ 69

Author(s):

John A. Jane ◽

Daniel M. Prevedello ◽

Tord D. Alden ◽

Edward R. Laws

Keyword(s):

Transsphenoidal Surgery ◽

Pediatric Patients ◽

Pediatric Population ◽

Case Series ◽

High Rate ◽

Subtotal Resection ◽

Consecutive Series ◽

Entire Cohort ◽

Transsphenoidal Resection

Object The majority of pediatric craniopharyngiomas are treated using a transcranial approach. Although there is an increasing acceptance of transsphenoidal resection in adults, there are few reports describing this approach in the pediatric population. The purpose of this study is to report the outcomes after transsphenoidal surgery in a consecutive series of pediatric patients with craniopharyngiomas treated at a single institution with the goal of gross-total resection (GTR). Methods Twenty-three patients with pathologically proven craniopharyngiomas were identified who were 18 years of age or less at the time of surgery. The medical records and imaging studies of the patients were retrospectively reviewed. One patient who was lost to follow-up after surgery was excluded. Results Among the 22 patients included in the study, 11 underwent transsphenoidal surgery as the primary procedure and 11 underwent transsphenoidal surgery as a secondary procedure after a previous procedure. All patients had at least some sellar component to their tumor and all had either anterior or posterior pituitary dysfunction at presentation. In the entire cohort, a GTR was achieved in 15 (68%) of 22 patients, a radical subtotal resection in 4 (18%) of 22 patients, a subtotal resection in 1 patient, and a partial resection in 2 patients. The degree of resection was higher in the primary transsphenoidal group. After a mean follow-up of 82 months, 4 patients (18%) experienced recurrence. Recurrence occurred in 13% after GTR compared with 28.5% after all other degrees of resection. Tumor recurred in 9% of the primary transsphenoidal group and in 30% of patients who had undergone other therapies prior to the transsphenoidal operation. No patient who had panhypopituitarism experienced a gain of function postoperatively, 67% developed new panhypopituitarism, and 56% experienced new diabetes insipidus. Vision improved or normalized in 9 (64%) of 14 patients presenting with visual loss. Complications included 1 death 3 weeks postoperatively, 2 CSF leaks, and new obesity in 37%. Conclusions Transsphenoidal resection of pediatric craniopharyngiomas results in a high rate of both visual improvement and GTR with a low associated risk of recurrence. The transsphenoidal approach should be considered in selected pediatric patients with craniopharyngioma, especially those with infradiaphragmatic origin.

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Outcomes of COVID-19 in a cohort of pediatric patients with rheumatic diseases

10.21203/rs.3.rs-145918/v1 ◽

2021 ◽

Author(s):

Diana Sofia Villacis-Nunez ◽

Christina A. Rostad ◽

Kelly Rouster-Stevens ◽

Arezou Khosroshahi ◽

Shanmuganathan Chandrakasan ◽

...

Keyword(s):

Rheumatic Disease ◽

Rheumatic Diseases ◽

Pediatric Patients ◽

Univariate Analysis ◽

Case Series ◽

Hospitalized Patients ◽

High Dose ◽

High Dose Corticosteroid ◽

Severe Immunosuppression ◽

Dose Corticosteroid

Abstract Background There are few reports of COVID-19 in pediatric patients with rheumatic diseases. This study describes the clinical presentation and outcomes of COVID-19 in this population. Methods We analyzed a single-center case series of pediatric patients with rheumatic diseases and laboratory-confirmed COVID-19. Demographic, baseline and COVID-19 associated clinical features were compared between ambulatory and hospitalized patients using univariate analysis. Results Forty cases were identified: 32 (80%) in the ambulatory group and 8 (20%) hospitalized. Older age (median age 18 years vs 16 years; p = 0.01) and African American race (OR 8.42; 95% CI [1.20-101.69]; p = 0.01) predominated in hospitalized patients. Systemic lupus erythematosus (OR 6.77; 95% CI [1.01–56.71]; p = 0.02), medium/high-dose corticosteroid use (OR 10.62; 95% CI [1.46–99.57]; p = 0.008), mycophenolate use (OR 11.91; 95% CI [1.64-149.35]; p = 0.005), and severe immunosuppression (OR 16.83; 95% CI [1.74-861.43]; p = 0.004) were associated with increased odds of hospitalization. Patients with fever (OR 11.91; 95% CI [1.64-149.35]; p = 0.004), dyspnea (OR 16.51; CI [1.10-998.37]; p = 0.02), and myalgias (OR 13.40; 95% CI [1.43-194.56)]; p = 0.009) were more commonly encountered in the hospitalized group. Rheumatic disease flares were almost exclusive to hospitalized patients (OR 42.13; 95% CI [3.40-2463.87]; p < 0.001).. All patients recovered. Conclusions Medium/high-dose corticosteroid use, mycophenolate use, and severe immunosuppression were risk factors for hospitalization. Fever, dyspnea and myalgias were high-risk symptoms. The type of rheumatic disease, as well as disease flare could be contributing factors to the need for hospitalization.

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First case series of the transradial approach for neurointerventional procedures in pediatric patients

Journal of Neurosurgery Pediatrics ◽

10.3171/2019.12.peds19448 ◽

2020 ◽

Vol 25 (5) ◽

pp. 492-496 ◽

Cited By ~ 1

Author(s):

Neil Majmundar ◽

Pratit Patel ◽

Vincent Dodson ◽

Ivo Bach ◽

James K. Liu ◽

...

Keyword(s):

Ultrasound Guidance ◽

Pediatric Patients ◽

Pediatric Population ◽

Case Series ◽

Transfemoral Approach ◽

Safe Alternative ◽

Transradial Approach ◽

Clinical Events ◽

First Case ◽

Neurointerventional Procedures

OBJECTIVEThe transradial approach (TRA) has been widely adopted by interventional cardiologists but is only now being accepted by neurointerventionalists. The benefits of the TRA over the traditional transfemoral approach (TFA) include reduced risk of adverse clinical events and faster recovery. The authors assessed the safety and feasibility of the TRA for neurointerventional cases in the pediatric population.METHODSPediatric patients undergoing cerebrovascular interventions since implementation of the TRA at the authors’ institution were retrospectively reviewed. Pertinent patient information, procedure indications, vessels catheterized, fluoroscopy time, and complications were reviewed.RESULTSThere were 4 patients in this case series, and their ages ranged from 13 to 15 years. Each patient tolerated the procedure performed using the TRA without any postprocedural issues, and only 1 patient experienced radial artery spasm, which resolved with the administration of intraarterial verapamil. None of the patients required conversion to the TFA.CONCLUSIONSThe TRA can be considered a safe alternative to the TFA for neurointerventional procedures in the pediatric population and provides potential advantages. However, as pediatric patients require special consideration due to their smaller-caliber arteries, routine use of ultrasound guidance is advised when attempting the TRA.

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Arthroscopic Management of Pigmented Villonodular Synovitis of the Hip in Children and Adolescents

Orthopaedic Journal of Sports Medicine ◽

10.1177/2325967118763118 ◽

2018 ◽

Vol 6 (3) ◽

pp. 232596711876311 ◽

Cited By ~ 3

Author(s):

S. Clifton Willimon ◽

Tim Schrader ◽

Crystal A. Perkins

Keyword(s):

Pediatric Patients ◽

Case Reports ◽

Pediatric Population ◽

Case Series ◽

Pigmented Villonodular Synovitis ◽

Villonodular Synovitis ◽

Arthroscopic Synovectomy ◽

Duration Of Symptoms ◽

Pigmented Villonodular

Background: Pigmented villonodular synovitis (PVNS) is a benign proliferative synovial disorder most commonly described to affect the knee in adults. Literature describing PVNS in the pediatric population is limited to 2 small case series and a handful of single-patient case reports. Within these studies, only 2 patients with PVNS of the hip are described. Purpose: To describe the presentation, management, and outcomes of a single-center series of pediatric patients with PVNS of the hip treated with arthroscopic synovectomy. Study Design: Case series; Level of evidence, 4. Methods: A retrospective review of consecutive pediatric patients treated for PVNS at a single institution was performed. Inclusion criteria consisted of patients younger than 19 years with surgically treated PVNS of the hip. Results: Five pediatric patients with a mean age of 11.0 years were treated for PVNS of the hip from 2011 to 2016. The mean duration of symptoms from onset to surgical treatment was 247 days (range, 3-933 days). Upon review of magnetic resonance imaging (MRI) results, radiologists included PVNS in their differential in 3 patients. Seven surgeries were performed in 5 patients. All therapeutic procedures were arthroscopic synovectomies. Nodular PVNS was present in 4 patients, and diffuse disease was present in 1 patient. At a mean 32-month follow-up (range, 12-63 months), all patients were considered to be free of recurrence based on clinical examination and/or follow-up MRI. Four patients were asymptomatic and returned to all of their previous sports activities. Conclusion: Young age at the time of diagnosis is a point to be highlighted in this cohort, and symptoms may be present for many months prior to diagnosis due to the failure to consider PVNS in children. Therefore, for patients with “atypical” presentations or lack of improvement with treatment for rheumatologic, bleeding, or infectious disorders, PVNS should be strongly considered. MRI with gradient echo sequences is the diagnostic imaging study of choice. One patient with diffuse involvement and preoperative degenerative changes showed progressive changes postoperatively. This type of PVNS may have a worse prognosis, but more diffuse cases are needed before the prognosis can be determined. Arthroscopic synovectomy following a timely diagnosis of PVNS produces good outcomes in nodular cases, with no evidence of symptomatic or radiographic disease persistence among these patients.

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Optical Coherence Tomography Imaging After Surgical Closure of Pediatric Traumatic Macular Holes

Journal of VitreoRetinal Diseases ◽

10.1177/2474126417749320 ◽

2018 ◽

Vol 2 (2) ◽

pp. 104-106 ◽

Cited By ~ 1

Author(s):

Nikisha Kothari ◽

Kimberly D. Tran ◽

Sarah P. Read ◽

Audina M. Berrocal

Keyword(s):

Optical Coherence Tomography ◽

Visual Acuity ◽

Macular Hole ◽

Pediatric Patients ◽

Surgical Repair ◽

Pediatric Population ◽

Case Series ◽

Optical Coherence ◽

Ellipsoid Zone ◽

Surgical Closure

Purpose: To date, little attention has been directed to retinal photoreceptor migration after traumatic macular hole surgical repair in the pediatric population. The purpose of this study was to evaluate optical coherence tomography (OCT) findings in the foveae of pediatric patients with history of traumatic macular hole surgical repair. Methods: Retrospective case series of 3 eyes in 3 pediatric patients with traumatic macular hole surgical repair. Spectral domain OCT images were obtained pre- and postoperatively. Results: We report 3 cases of restoration of the ellipsoid zone with good visual acuity outcomes following macular hole closure. Conclusion: Optical coherence tomography findings of an intact ellipsoid zone may be a predictor of visual acuity and explain better surgical outcomes in the pediatric population.

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Dural venous sinus stenting for treatment of pediatric idiopathic intracranial hypertension

Journal of NeuroInterventional Surgery ◽

10.1136/neurintsurg-2020-016183 ◽

2020 ◽

pp. neurintsurg-2020-016183

Author(s):

Katriel E Lee ◽

Aqib Zehri ◽

Sauson Soldozy ◽

Hasan Syed ◽

Joshua S Catapano ◽

...

Keyword(s):

Intracranial Hypertension ◽

Idiopathic Intracranial Hypertension ◽

Pediatric Patients ◽

Pediatric Population ◽

Case Series ◽

Venous Sinus ◽

Retrospective Case ◽

Safety And Efficacy ◽

Dural Venous Sinus

BackgroundDural venous sinus stenting (VSS) is an effective treatment for idiopathic intracranial hypertension (IIH) in adult patients. There are no published series to date evaluating safety and efficacy of VSS in pediatric patients.ObjectiveTo report on procedural device selection and technique as well as safety and efficacy of VSS for pediatric patients with medically refractory IIH due to underlying venous sinus stenosis.MethodsA multi-institutional retrospective case series identified patients with medically refractory IIH aged less than 18 years who underwent VSS.Results14 patients were identified at four participating centers. Patient ages ranged from 10 to 17 years, and 10 patients (71.4%) were female. Mean body mass index was 25.7 kg/m2 (range 15.8–34.6 kg/m2). Stenting was performed under general endotracheal anesthesia in all except two patients. The average trans-stenotic gradient during diagnostic venography was 10.6 mm Hg. Patients had stents placed in the superior sagittal sinus, transverse sinus, sigmoid sinus, occipital sinus, and a combination. Average follow-up was 1.7 years after stenting. Six patients out of 10 (60%) had reduced medication dosing, 12 of 14 patients (85.7%) had improvements in headaches, two patients (100%) with pre-stent tinnitus had resolution of symptoms, and four (80%) of five patients with papilledema had improvement on follow-up ophthalmological examinations. Two patients (14.3%) developed postprocedural groin hematomas, one patient (7.1%) developed a groin pseudoaneurysm, and one patient (7.1%) had postprocedural groin bleeding. No other procedural complications occurred. Four patients (28.6%) required further surgical treatment (cerebrospinal shunting and/or stenting) after their first stenting procedure.ConclusionsThis series suggests that VSS is feasible in a pediatric population with IIH and has a low complication rate and good clinical outcomes.

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Ocular manifestations of ectodermal dysplasia

Orphanet Journal of Rare Diseases ◽

10.1186/s13023-021-01824-2 ◽

2021 ◽

Vol 16 (1) ◽

Author(s):

Daphna Landau Prat ◽

William R. Katowitz ◽

Alanna Strong ◽

James A. Katowitz

Keyword(s):

Pediatric Patients ◽

Ectodermal Dysplasia ◽

Pediatric Population ◽

Case Series ◽

Sweat Glands ◽

Retrospective Case ◽

Ocular Manifestations ◽

Ectodermal Dysplasias ◽

New Finding ◽

Lacrimal Drainage

Abstract Purpose The ectodermal dysplasias (EDs) constitute a group of disorders characterized by abnormalities in two or more ectodermal derivatives, including skin, hair, teeth, and sweat glands. The purpose of the current study was to evaluate ocular manifestations in pediatric patients with ED. Methods Retrospective case series including consecutive ED subjects who were treated in the ophthalmology department at the Children’s Hospital of Philadelphia over a 12-year period (2009–2020). Main Outcome Measures were ocular and ocular adnexal abnormalities. Results Thirty subjects were included: 20 males (67%), mean age of 4.5 years (range 0.3–18). Patients with different subtypes were included, with the hypohidrotic ED and ectrodactyly-ectodermal dysplasia-clefting variants being most prevalent. Most common findings were: lacrimal drainage obstruction in 12 (40%) including punctal agenesis in 10 (33%), refractive errors in 13 (43%) and amblyopia in 6 (20%). A new finding of eyelid ptosis or eyelash ptosis was demonstrated in 11 subjects (37%), mostly associated with TP63 or EDA1 genes variants. Conclusion Ectodermal dysplasias are associated with various ocular pathologies and amblyopia in the pediatric population. We report a possible genetic association between lash ptosis and EDA1 gene, and eyelid ptosis and TP63 or EDA1 genes variants.

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Acute Intoxication Caused by a Synthetic Cannabinoid in Two Adolescents

The Journal of Pediatric Pharmacology and Therapeutics ◽

10.5863/1551-6776-17.2.177 ◽

2012 ◽

Vol 17 (2) ◽

pp. 177-181 ◽

Cited By ~ 14

Author(s):

Travis S. Heath ◽

Zachary Burroughs ◽

A. Jill Thompson ◽

Frederick W. Tecklenburg

Keyword(s):

Adverse Effects ◽

Pediatric Patients ◽

Pediatric Population ◽

Synthetic Cannabinoids ◽

Case Series ◽

Marijuana Use ◽

Altered Mental Status ◽

Synthetic Cannabinoid ◽

Myocardial Infarctions ◽

Loss Of Consciousness

Illicit drug use continues to be a common problem among pediatric patients. Daily marijuana use among high school seniors is currently at a 30-year high. Marijuana use in adults has rarely been associated with cardiovascular adverse effects, including hypertension, tachycardia, arrhythmia, and myocardial infarction. Recently, abuse of synthetic cannabinoids, such as the incense “K2” or “Spice,” has been increasingly reported in the lay press and medical literature. Overdose and chronic use of these substances may cause adverse effects including altered mental status, tachycardia, and loss of consciousness. Overdoses in adult patients have been described; however, limited reports in the pediatric population have been documented. A recent case series describes myocardial infarctions in pediatric patients, associated with synthetic cannabinoid use. In this report, we describe two adolescent patients admitted after they inhaled “K2,” resulting in loss of consciousness, tachycardia, and diffuse pain.

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