Male Pediatric Patients Research Articles

Hematologic parameters as predictive markers in pediatric Bell's palsy.

To analyze various hematological parameters in pediatric population with Bell's palsy (BP) and to determine the most valuable parameter as a prognostic marker. Fifty-nine patients with BP under 18years were enrolled, and patients were divided into three groups: recovery group (49 cases), non-recovery group (10 cases) and controls (65 healthy children). Age, white blood cell (WBC), hemoglobin (Hbg), mean platelet volume (MPV), red cell distribution width (RDW), neutrophil-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR) and C-reactive protein-to-albumin ratio (CAR) were determined and compared between groups. Additionally, the receiver operating characteristics (ROC) analysis was carried out, and the most valuable marker to demonstrate the prognosis among these parameters was investigated. While mean age was found as 10.69 ± 5.76years, 28 (47.5%) female and 31 (52.5%) male pediatric patients were determined to be followed up due to BP in our hospital. When such parameters age, mean Hbg, MPV and RDW were examined, no significant difference was detected between groups. However, compared controls with recovery and non-recovery groups, WBC, NLR, PLR and CAR were found to be significantly higher, respectively (p < 0.05). Median CAR and NLR values were found to be significantly higher in non-recovery group, compared to those in recovery group (p = 0.001, p = 0.012, respectively). However, when median WBC and PLR were examined between recovery and non-recovery groups, no significant difference was observed (p > 0.05). High NLR and CAR values in pediatric BP may be related to poor prognosis in such patients. CAR, however, is a more valuable parameter than NLR in terms of indicating poor prognosis.

Use of stainless steel crowns and zirconia crowns in posterior primary teeth for children in different age groups

Children are more susceptible to Early Childhood Caries and trauma. Post endodontic treatment, it is necessary for coverage of the treated teeth, and full crowns are considered to be the best option for the same. Preformed crowns for primary molars are available in many sizes and materials to be placed over decayed or developmentally defective teeth. They can be made completely of stainless steel, stainless steel with a white veneer cover or made wholly of a white ceramic material when aesthetics is concerned. The main aim of this study is to assess the prevalence of usage of stainless steel crowns and zirconia crowns in posterior teeth for pedo patients in different age groups. 89000 case sheets were reviewed between June 2019 - March 2020 for possible inclusion and exclusion criteria. Case sheets of 1496 pediatric patients who underwent stainless steel crown and zirconia crown placement in posterior teeth were obtained from a Private Dental Institute. Collected data were analysed using SPSS statistics software. Results showed that stainless steel crowns were more prevalently used among all age groups (99.54%) and were more prevalent among the age group 0-5 years (69.61%). Stainless steel crowns were also found to be more prevalent among male pediatric patients (57.80%). In Conclusion, stainless steel crowns were most prevalent among all age groups and are more prevalent among male pediatric patients.

Comparison of Simultaneous Capillary Blood Gases and End-Tidal Carbon Dioxide in Mechanically Ventilated Pediatric Patients with Acute Respiratory Failure.

In the follow-up of ventilation, invasive blood gas analysis and noninvasive monitoring of end-tidal carbon dioxide (ETCO 2 ) are used. We aimed to investigate the relationship between capillary partial pressure of carbon dioxide (PcCO 2 ) levels and ETCO 2 and also to investigate ETCO 2 's predictive feature of PcCO 2 levels. This study included 28 female and 30 male pediatric patients; 28 patients were type-1 respiratory failure (RF), 16 patients were acute respiratory distress syndrome, and 14 patients were type-2 RF. Our results showed a significant correlation between ETCO 2 and PcCO 2 . Although the strength of the correlation was weak throughout the measurements, the strength of this correlation increased significantly in type-2 RF.

Temporary Central Vision Blindness After Oseltamivir Administration in a 15-Year-Old Pediatric Male Positive for Influenza A.

Objective: A 15-year-old pediatric male patient was influenza A positive and started on oseltamivir at an outpatient clinic. Method: The next morning the patient presented to the emergency department (ED) with a chief complaint of visual disturbances including decreased central vision. Prior to presenting to the ED the patient was evaluated by his optometrist and his eye exam tested 20/400 bilaterally. His previous year's eye exam was normal, 20/25 bilaterally. Results: In the ED, the patient had an MRI which showed a normal appearing optic nerve, chiasm, and optic tracts. The oseltamivir therapy was discontinued, and the patient followed up with an ophthalmologist outpatient. Conclusion: At a 10-week follow-up visit the patient had 90% recovery of his vision.

Serum Ferritin Correlates With Liver Fat in Male Adolescents With Obesity.

Non-alcoholic fatty liver disease (NAFLD) contributes essentially to the burden of obesity and can start in childhood. NAFLD can progress to cirrhosis and hepatocellular carcinoma. The early phase of NAFLD is crucial because during this time the disease is fully reversible. Pediatric NAFLD shows unique features of histology and pathophysiology compared to adults. Changes in serum iron parameters are common in adult NAFLD and have been termed dysmetabolic iron overload syndrome characterized by increased serum ferritin levels and normal transferrin saturation; however, the associations of serum ferritin, inflammation, and liver fat content have been incompletely investigated in children. As magnetic resonance imaging (MRI) is an excellent measure for the degree of liver steatosis, we applied this method herein to clarify the interaction between ferritin and fatty liver in male adolescents. For this study, one hundred fifty male pediatric patients with obesity and who are overweight were included. We studied a subgroup of male patients with (n = 44) and without (n = 18) NAFLD in whom we determined liver fat content, visceral adipose tissue, and subcutaneous adipose tissue extent with a 1.5T MRI (Philips NL). All patients underwent a standardized oral glucose tolerance test. We measured uric acid, triglycerides, HDL-, LDL-, total cholesterol, liver transaminases, high sensitive CRP (hsCRP), interleukin-6, HbA1c, and insulin. In univariate analysis, ferritin was associated with MRI liver fat, visceral adipose tissue content, hsCRP, AST, ALT, and GGT, while transferrin and soluble transferrin receptor were not associated with ferritin. Multivariate analysis identified hsCRP and liver fat content as independent predictors of serum ferritin in the pediatric male patients. Our data indicate that serum ferritin in male adolescents with obesity is mainly determined by liver fat content and inflammation but not by body iron status.

Outcomes of Dog Bite Avulsion Injury Reconstruction With Urinary Bladder Matrix.

Dog bite avulsion injuries of the head and neck are difficult to manage in pediatric patients. This study assesses the outcomes of using porcine urinary bladder extracellular matrix (UBM) for reconstruction of these complete avulsion injuries. Five male pediatric patients underwent reconstruction using UBM. Two (40%) patients underwent reconstruction of the nose; the other 3 patients underwent reconstruction of the forehead, forehead/glabella, and auricle. The average size of the avulsion defect was 7.0 ± 2.4 cm2. No patient developed wound dehiscence, graft loss, or wound infection. Four (80%) patients received pulsed dye laser treatment to improve wound cosmesis. Use of UBM is a safe and effective reconstructive option after dog bite avulsion injuries of the head and neck. Given the advantages of convenient availability and avoidance of donor site morbidity, UBM can be considered for reconstruction of posttraumatic avulsion injuries or Mohs defects.

A COMPARISON BETWEEN TRANSURETHRAL CYSTOLITHOCLASTY AND VESICOLITHOTOMY IN PEDIATRIC MALE PATIENTS

ABSTRACT&#x0D; BACKGROUND &amp; OBJECTIVE: To compare transurethral cystolithoclasty with ureteroscope over open vesicolithotomy in pediatric male patients.&#x0D; METHODOLOGY: Study comprises of hundred pediatric male patients, divided into two equal groups of 50 each, labeled as Group-A and Group-B. All the patients having stones less than 20mm size (average 14mm) were included. Group-A patients were treated with transurethral cystolithoclasty by using ureteroscope and pneumatic lithoclast assisted with the help of Dormia basket. Group-B patients were treated with open vesicolithotomy.RESULTS: All hundred male pediatric patients were between the ages of 1-15 years (mean age 8 years). Hospital stay was 2-3 days (average 2.5 days) in Group-A patients and 3-5 days (average 4 days) in Group-B patients. In Group-A3 (6%) patients had urinary tract infection, 1(2%) had urethral injury and 2(4%) had residual stone post-operatively. While Group-B4 (8%) suffered from urinary tract infection, 1(2%) had wound infection, 1(2%) had hematuria, and 2(4%) had fever. Retention of urine was 1(2%) in each Group.&#x0D; CONCLUSION: Transurethral cystolithoclasty is a much better way of treating vesical calculus in pediatric male patients because it is a procedure with short hospital stay, minimal invasion, no scar and less complication. KEYWORDS: Bladder Calculus, Comparison, Cystolithoclasty, Ureteroscope, Pneumatic Lithoclast, Dormia basket, Vesicolithotomy.

P3BEP (ANZUP 1302): An international randomized phase III trial of accelerated versus standard BEP chemotherapy for adult and pediatric male and female patients with intermediate and poor-risk metastatic germ cell tumors (GCTs).

TPS425 Background: Bleomycin, etoposide, cisplatin (BEP) given 3-weekly x 4 remains standard 1st line chemotherapy for metastatic GCTs. Acceleration of standard regimen with shorter cycle lengths has improved cure rates in other cancers. This is the first international randomised clinical trial for intermediate and poor-risk metastatic extracranial GCTs involving both adult and paediatric age group males and females. We aim to determine if accelerated BEP is superior to standard BEP. Methods: DESIGN: Open label, randomised, stratified multicentre, 2 stage, phase 3 trial. Primary endpoint for stage I (n = 150) is the achievement of a favourable response, and for entire trial (n = 500) is progression free survival (PFS). SAMPLE SIZE: 150 and 500 patients gives &gt; 80% power to detect a 20% improvement in the favourable response rate and 7% absolute improvement in 2yr PFS, respectively. POPULATION: Males and females aged 11-45 years with intermediate or poor-risk metastatic GCTs of the testis, ovary, retroperitoneum or mediastinum for 1st line chemotherapy. TREATMENT: Randomisation 1:1 to 4 cycles of “standard BEP” or “accelerated BEP”: cisplatin 20mg/m2 IV D1-5; etoposide 100mg/m2 IV D1-5; bleomycin 30000 IU IV weekly; and pegylated G-CSF SC D6 or filgrastim daily; given every 3 weeks or every 2 weeks respectively. Accelerated BEP arm receives 4 additional weekly doses of bleomycin. ASSESSMENTS: Response assessments at 30 day safety assessment, and 6 months from randomisation or after all post-chemotherapy intervention is completed. Regular follow-up up to 5 years, then annually. Archival tumour tissue and baseline blood collected for translational substudies. STATUS: 25 sites open in ANZ, 13 sites open in UK (led by Cambridge Clinical Trials Unit), 67 sites open in the USA (led by Children’s Oncology Group). As of September 2019, 98 patients have been recruited triggering the first formal interim analysis. Clinical trial information: NCT02582697.

Pediatric Maxillofacial Trauma Management Experience at Tertiary Care Hospital- A Retrospective Study

ABSTRACT Objectives: To establish the etiology, pattern, management and outcome of maxillofacial trauma in pediatric patients. Study Design: Retrospective study. Setting and Duration: Department of Oral & Maxillofacial Surgery, Faisalabad Medical University, Faisalabad. January 2016 to January 2019. Materials and Methods: A three year record of pediatric patients who suffered from maxillofacial trauma was reviewed and analyzed using hospital database. The data gathered from the record of the patient’s like age which was presented as mean and standard deviation whereas data like gender, etiology of particular trauma, anatomic position of fracture, affiliated injuries, kind of treatment used and outcome of the treatment was presented as frequency and percentage. Results: Out of 135 pediatric maxillofacial trauma patients 91 (67.4%) were males and 44 (32.6%) were females making male to female ratio of 1.8:1. The mean age of the pediatric patients was 6.5 + 2.63 years. Fall was the most common etiology of the trauma 83 (61.5%) followed by RTA 40 (29.6%). Out of 157 different fracture types 113 (71.9%) occurred in mandible, and among mandible parasymphysis region was most commonly 42 (37.1%) fractured site. Majority of the patients were treated by ORIF 80 (59.3%). The concomitant injuries were observed in 31 (22.9%) patients and among these neuro-cranial injuries were most common in 19 (61.3%) patients. Post treatment complications were observed in 16 (11.9%) patients and among these limited mouth opening was the most commonly seen complication 8 (50%) patients. Conclusion: The results of this study verified pediatric male patient’s predominance. Mandible fracture was the most frequent site of pediatric maxillofacial trauma, predominantly caused by a fall. Majority of maxillofacial fractures were managed by open reduction and internal fixation. There is a need at a national level for a public, parent and teacher education program on child-care and safety.

Female adolescents demonstrate greater oculomotor and vestibular dysfunction than male adolescents following concussion

ObjectiveTo examine differences between male and female adolescents on measures of balance, vestibular and oculomotor function within 3 weeks of concussion among a group of pediatric patients presenting to a sports medicine clinic. DesignMedical record review. SettingSports medicine clinic. Participants197 female (median age = 15.2 years) and 381 male (median age = 14.6 years) pediatric patients seen for a concussion, evaluated 9.3 ± 5.2 (mean ± SD) days post-concussion. Main outcome measuresPatients completed Balance Error Scoring System (BESS), tandem gait, gaze stability, and near point of convergence (NPC) tests. ResultsA higher proportion of female patients exhibited abnormal NPC (22% vs. 14%; p = 0.017), gaze stability (53% vs. 43%; p = 0.028), and tandem gait tests (20% vs. 13%; p = 0.026) compared to male patients. Multivariable analysis indicated an independent association between female sex and increased odds of abnormal NPC (adjusted odds ratio [aOR] = 1.79, 95% CI = 1.07–3.00), and tandem gait tests (aOR = 1.96, 95% CI = 1.12–3.41) following concussion. ConclusionsOur results indicate that within the first three weeks of a concussion, female pediatric patients demonstrated increased odds of exhibiting abnormal near point of convergence, and tandem gait test performance compared to male patients.

Primary lymphangioma circumscriptum around external urethral meatus in a male child: A rare presentation

Primary lymphangioma circumscriptum (LC) is a superficial lymphatic malformation, presenting as multiple fluid-filled grouped vesicular lesions which may be associated with slight hemorrhages. We report a rare case of primary LC with exclusive involvement of area around the external urethral meatus in a 10-year-old male child. We treated the patient with radiofrequency ablation under topical anesthesia with eutectic mixture of local anesthetics (EMLA). No recurrence has been observed in the past 1 year. LC must be included in differentials when presented with vesicular lesions near the external urethral meatus in male pediatric patients.

Current Clinical Practice About Pediatric Midline Gliomas in the Scope of Molecular Era.

To share our clinical experience with surgical and adjuvant treatment strategies followed during the treatment of midline gliomas. Pediatric patients with midline gliomas who underwent surgery in our clinic between March 2016 and November 2019 were included. Tissue samples were obtained through surgical excision, open biopsy, or stereotactic biopsy. All samples were analyzed for ATRX, BRAFV600E, IDH1/2, H3K27M, and H3G34R mutations, EGFR and PGFRA amplifications, and PTEN loss. There were 7 (43.8%) female and 9 (56.2%) male pediatric patients in the study. Eight patients had thalamic, 5 patients had pontine, 2 patients had medulla oblongata and one patient had brachium pontis tumors. Presenting symptoms were headache, disequilibrium, ophthalmoplegia, and panic attack. Eleven tumors showed H3K27M mutation and were diagnosed as diffuse midline gliomas. BRAFV600E, ATRX mutations, PTEN loss, and EGFR amplifications were other molecular alterations detected within tumor samples. Patients with H3K27M mutant tumors had a shorter life span. Five patients were enrolled in an ONC201 trial. Although most midline gliomas are not amenable to gross total excision, obtaining tissue samples is mandatory for determining patients? exact diagnoses, tailored treatment plans, and eligibility for clinical trials. Stereotactic biopsy for midline gliomas is a safe and effective method.

Ultrasound-guided nerve block for pediatric inguinal herniorrhaphy

ABSTRACT Background and aim Regional anesthesia is one of the best options anesthetic technique, it was very difficult to be used in pediatrics anesthesia, now a days it becomes more easier and safer with the era of high-quality ultrasound. This study aimed to assess the effectiveness of ultrasound-guided nerve block (ilioinguinal/iliohypogastric; II/IH) in pediatric unilateral inguinal herniorraphy, time for first analgesic dose, parents, surgeon satisfaction and complication. Patients and methods The study was done in Zagazig university hospital after approval of the ethical committee. Induction of anesthesia using sevoflorane MAC (Minimal Alveolar Concentration) 4%-6% then Laryngeal Mask Airway was inserted (LMA). Ultrasound-guided (ilioinguinal/iliohypogastric) nerve block was performed on 20 male pediatric patients their age ranged from 4 to 10 years old with ASA status I and II, with unilateral inguinal hernia.hemodynamics as heart rate (HR) blood pressure systolic/diastolic (SBP/DBP) was reported also Children Infants Postoperative Pain (CHIPPS) score was recorded every 2 h until 12 h and time for first analgesic dose also reported. Results Our result showed no significant changes in heart rate (HR) and blood pressure (BP) at skin incision; HR (95 ± 8), BP (97.6±/50 ± 5) and intraoperative HR (93.5 ± 6), BP (99.6 ± 9/51 ± 4) compared with the basal readings; HR (113 ± 10), BP (104 ± 12/53 ± 6). Pain score was evaluated using (ChIPPS), it started to increase after 4 to 5 h and reported by first analgesic dose (5.2 ± 1.5) that managed by paracetamol (15 mg/kg/day). Surgeon and parents were satisfied. Early ambulation and less hospital stay. Less complications (no motor block or urine retention). Conclusion Ultrasound-guided (ilioinguinal and iliohypogastric) nerve block was found to be an ideal intraoperative anesthetic and postoperative analgesic for unilateral inguinal herniorrhaphy in children with no complications.

Are demographics associated with mucoepidermoid or acinic cell carcinoma parotid malignancies in children?

ObjectiveTo identify possible associations between patient demographics and parotid cancer histological type in pediatric patients.MethodsPediatric patients (ages: birth-18.0 years) in the Surveillance, Epidemiology, and End Results (SEER) database were included from 1973 to 2014 based on a diagnosis of mucoepidermoid carcinoma or acinic cell carcinoma of the parotid gland using the ICD O-3 codes of C07.9 and 8430 or 8550. Patients were classified into the following cohorts: <14 and 14–18 years of age based on the mean age at diagnosis.ResultsThree hundred and three pediatric patients were diagnosed with mucoepidermoid carcinoma or acinic cell carcinoma of the parotid gland within the SEER 18 registries. Female pediatric patients 14–18 years of age were 7.68 times more likely to have an acinic cell carcinoma (adjusted OR: 7.68 [95% CI: 2.01–29.44]). When stratified by histological type, 58.9% of female pediatric patients ≥14 years of age had an acinic cell carcinoma as compared to 37.3% of male pediatric patients ≥14 years of age, 36.5% of female pediatric patients <14 years of age, and 34.0% of male pediatric patients <14 years of age (P = 0.01).ConclusionsBased on this study, pediatric female patients between the ages of 14 and 18 years are the most likely cohort to have acinic cell carcinoma. The results of this study may assist providers during the work up of a pediatric patient with a suspected parotid malignancy.

Evaluation of the Cases Hospitalized in our Burn Unit

Aim: A burn is a physiopathological process where damage occurs in the skin and subcutaneous tissues and can affect the entire body depending on its depth and surface area and the reason causing the burn. Burns are most common in children and in the home environment according to a large number of studies. We compared the clinical and demographic features and the treatment protocols of our cases who were diagnosed with a burn in this study. Material and Methods: A total of 52 patients who presented to the Erzincan State Hospital's Burn Unit between July 2008 and June 2010 and were diagnosed with a burn were included in our study. The charts of our cases were retrospectively evaluated. The demographic findings, burn surface area and burn degree determined during the physical examination were recorded from the charts. Results The group of 52 subjects presenting to the burn unit consisted of 33 pediatric and 19 adult patients. The mean age was 18.2 years. There were 17 female and 16 male pediatric patients. The adult patients consisted of 12 females and 7 males. A first-degree burn with an area between 10% to 15% of the body surface was found in 12 of our pediatric patients and 8 of our adult patients. All the burns were hot water burns or sunburns. These subjects were prescribed medical treatment for one week and were referred to the outpatient department where the follow-up was performed. The mean duration until recovery was 7 to 10 days. A second-degree burn with a surface area between 25% and 40% was found in 15 pediatric patients and 6 adult patients. All these burns had been caused by hot water. Our cases were admitted to the burn unit and treated by dressing the wound. The mean duration until recovery was 4 to 6 weeks. The patients were discharged with full recovery and without any complications. A third-degree burn was found in 6 (18.18%) of our pediatric patients and in 4 (21.05%) of our adult patients. The patients were admitted to the burn unit and dressings alone or with debridement were used for treatment according to the burn healing status. Conclusion Burn patients should be evaluated in detail. Deepening of the burn, infection and fluid loss can be prevented by appropriate intervention in the early period. The appropriate treatment plan should be arranged and the patients who require treatment at a burn center should be referred. DOI: 10.7176/JSTR/5-12-17

BRAF V600E mutation in Juvenile Xanthogranuloma family neoplasms of the central nervous system (CNS-JXG): a revised diagnostic algorithm to include pediatric Erdheim-Chester disease

The family of juvenile xanthogranuloma family neoplasms (JXG) with ERK-pathway mutations are now classified within the “L” (Langerhans) group, which includes Langerhans cell histiocytosis (LCH) and Erdheim Chester disease (ECD). Although the BRAF V600E mutation constitutes the majority of molecular alterations in ECD and LCH, only three reported JXG neoplasms, all in male pediatric patients with localized central nervous system (CNS) involvement, are known to harbor the BRAF mutation. This retrospective case series seeks to redefine the clinicopathologic spectrum of pediatric CNS-JXG family neoplasms in the post-BRAF era, with a revised diagnostic algorithm to include pediatric ECD. Twenty-two CNS-JXG family lesions were retrieved from consult files with 64% (n = 14) having informative BRAF V600E mutational testing (molecular and/or VE1 immunohistochemistry). Of these, 71% (n = 10) were pediatric cases (≤18 years) and half (n = 5) harbored the BRAF V600E mutation. As compared to the BRAF wild-type cohort (WT), the BRAF V600E cohort had a similar mean age at diagnosis [BRAF V600E: 7 years (3–12 y), vs. WT: 7.6 years (1–18 y)] but demonstrated a stronger male/female ratio (BRAF V600E: 4 vs WT: 0.67), and had both more multifocal CNS disease ( BRAFV600E: 80% vs WT: 20%) and systemic disease (BRAF V600E: 40% vs WT: none). Radiographic features of CNS-JXG varied but typically included enhancing CNS mass lesion(s) with associated white matter changes in a subset of BRAF V600E neoplasms. After clinical-radiographic correlation, pediatric ECD was diagnosed in the BRAF V600E cohort. Treatment options varied, including surgical resection, chemotherapy, and targeted therapy with BRAF-inhibitor dabrafenib in one mutated case. BRAF V600E CNS-JXG neoplasms appear associated with male gender and aggressive disease presentation including pediatric ECD. We propose a revised diagnostic algorithm for CNS-JXG that includes an initial morphologic diagnosis with a final integrated diagnosis after clinical-radiographic and molecular correlation, in order to identify cases of pediatric ECD. Future studies with long-term follow-up are required to determine if pediatric BRAF V600E positive CNS-JXG neoplasms are a distinct entity in the L-group histiocytosis category or represent an expanded pediatric spectrum of ECD.

Effect of Graft Type on Thigh Circumference, Knee Range of Motion, and Lower-Extremity Strength in Pediatric and Adolescent Males Following Anterior Cruciate Ligament Reconstruction.

To treat anterior cruciate ligament (ACL) injury, ACL reconstruction (ACLR) surgery is currently a standard of the care. However, effect of graft type including bone-patellar tendon-bone (BTB), hamstring tendon, or iliotibial band (ITB) on thigh size, knee range of motion (ROM), and muscle strength are understudied. To compare postoperative thigh circumference, knee ROM, and hip and thigh muscle strength in adolescent males who underwent ACLR, based on the 3 different autograft types: BTB, hamstring (HS), and ITB. Biomechanical laboratory. Male ACLR patients who are younger than 22 years of age (total N = 164). At 6- to 9-month postoperative visits, thigh circumference, knee ROM, and hip and thigh muscle strength were measured. Deficits of each variable between the uninvolved and ACLR limb were compared for pediatric and adolescent ACLR males in the BTB, HS, and ITB cohorts. Baseline characteristics, including physical demographics and meniscus tear status, were compared, and differences identified were treated as covariates and incorporated in analysis of covariance. Data were from 164 adolescent male ACLR patients [mean age 15.7 (1.2) years]. There were no statistical differences in thigh circumference, knee ROM, hip abductor, and hip-extensor strength among the 3 autografts. However, patients with BTB demonstrated 12.2% deficits in quadriceps strength compared with 0.5% surplus in HS patients (P = .002) and 1.2% deficits in ITB patients (P = .03). Patients with HS showed 31.7% deficits in hamstring strength compared with 5.4% deficits in BTB (P = .001) and 7.7% deficits in ITB (P = .001) groups at 6- to 9-month postoperative visits. Adolescent male ACLR patients with BTB and HS autografts demonstrated significant deficits in quadriceps and hamstring strength, respectively, at 6 to 9 months postoperatively. Minimal lower-extremity strength deficits were demonstrated in pediatric male ACLR patients undergoing ITB harvest.

Effectiveness and safety of the tri-iodothyronine analogue Triac in children and adults with MCT8 deficiency: an international, single-arm, open-label, phase 2 trial

Deficiency of the thyroid hormone transporter monocarboxylate transporter 8 (MCT8) causes severe intellectual and motor disability and high serum tri-iodothyronine (T3) concentrations (Allan-Herndon-Dudley syndrome). This chronic thyrotoxicosis leads to progressive deterioration in bodyweight, tachycardia, and muscle wasting, predisposing affected individuals to substantial morbidity and mortality. Treatment that safely alleviates peripheral thyrotoxicosis and reverses cerebral hypothyroidism is not yet available. We aimed to investigate the effects of treatment with the T3 analogue Triac (3,3',5-tri-iodothyroacetic acid, or tiratricol), in patients with MCT8 deficiency. In this investigator-initiated, multicentre, open-label, single-arm, phase 2, pragmatic trial, we investigated the effectiveness and safety of oral Triac in male paediatric and adult patients with MCT8 deficiency in eight countries in Europe and one site in South Africa. Triac was administered in a predefined escalating dose schedule-after the initial dose of once-daily 350 μg Triac, the daily dose was increased progressively in 350 μg increments, with the goal of attaining serum total T3 concentrations within the target range of 1·4-2·5 nmol/L. We assessed changes in several clinical and biochemical signs of hyperthyroidism between baseline and 12 months of treatment. The prespecified primary endpoint was the change in serum T3 concentrations from baseline to month 12. The co-primary endpoints were changes in concentrations of serum thyroid-stimulating hormone (TSH), free and total thyroxine (T4), and total reverse T3 from baseline to month 12. These analyses were done in patients who received at least one dose of Triac and had at least one post-baseline evaluation of serum throid function. This trial is registered with ClinicalTrials.gov, number NCT02060474. Between Oct 15, 2014, and June 1, 2017, we screened 50 patients, all of whom were eligible. Of these patients, four (8%) patients decided not to participate because of travel commitments. 46 (92%) patients were therefore enrolled in the trial to receive Triac (median age 7·1 years [range 0·8-66·8]). 45 (98%) participants received Triac and had at least one follow-up measurement of thyroid function and thus were included in the analyses of the primary endpoints. Of these 45 patients, five did not complete the trial (two patients withdrew [travel burden, severe pre-existing comorbidity], one was lost to follow-up, one developed of Graves disease, and one died of sepsis). Patients required a mean dose of 38.3 μg/kg of bodyweight (range 6·4-84·3) to attain T3 concentrations within the target range. Serum T3 concentration decreased from 4·97 nmol/L (SD 1·55) at baseline to 1·82 nmol/L (0·69) at month 12 (mean decrease 3·15 nmol/L, 95% CI 2·68-3·62; p<0·0001), while serum TSH concentrations decreased from 2·91 mU/L (SD 1·68) to 1·02 mU/L (1·14; mean decrease 1·89 mU/L, 1·39-2·39; p<0·0001) and serum free T4 concentrations decreased from 9·5 pmol/L (SD 2·5) to 3·4 (1·6; mean decrease 6·1 pmol/L (5·4-6·8; p<0·0001). Additionally, serum total T4 concentrations decreased by 31·6 nmol/L (28·0-35·2; p<0·0001) and reverse T3 by 0·08 nmol/L (0·05-0·10; p<0·0001). Seven treatment-related adverse events (transiently increased perspiration or irritability) occurred in six (13%) patients. 26 serious adverse events that were considered unrelated to treatment occurred in 18 (39%) patients (mostly hospital admissions because of infections). One patient died from pulmonary sepsis leading to multi-organ failure, which was unrelated to Triac treatment. Key features of peripheral thyrotoxicosis were alleviated in paediatric and adult patients with MCT8 deficiency who were treated with Triac. Triac seems a reasonable treatment strategy to ameliorate the consequences of untreated peripheral thyrotoxicosis in patients with MCT8 deficiency. Dutch Scientific Organization, Sherman Foundation, NeMO Foundation, Wellcome Trust, UK National Institute for Health Research Cambridge Biomedical Centre, Toulouse University Hospital, and Una Vita Rara ONLUS.

Optimal Predictor of Gonadal Viability in Testicular Torsion: Time to Treat Versus Duration of Symptoms

BackgroundWe hypothesize that in testicular torsion, the duration of symptoms (DoS) better correlates with predicting testicular viability than minimizing the “time-to-treat” (TtT) after presentation to a medical facility. Materials and methodsMedical records of male pediatric patients treated for suspected diagnosis of testicular torsion in the emergency department (ED) from January 1, 2016, to December 31, 2018, were retrospectively evaluated. Forty-one patients met inclusion criteria. Statistical analysis compared testicular viability based on TtT, DoS, and site of initial presentation. ResultsTesticular salvage rates for patients presenting directly to our ED was 56.3% with an average TtT of 2.5 h versus 77.8% and 1.96 h, respectively, for transferred patients. Overall testicular survival was not statistically impacted by the difference in TtT. Comparing DoS, an 84% testicular salvage rate (DoS < 24 h) versus a 15.4% salvage rate (DoS > 24 h) was shown in patients presenting directly to our ED (P ≤ 0.0001). Within the total population (n = 41), a significant difference was also shown (P ≤ 0.0001) when comparing overall testicular salvage rates in patients presenting with <24 h versus >24 h total DoS (84% versus 25%). ConclusionsThese data reveal that an alternative predictor of testicular salvage rates is a DoS < 24 h. This is a meaningful metric when providing accurate preoperating counseling to parents and may be a better focus of quality improvement efforts surrounding this topic.

A rare form of ion channel gene mutation identified as underlying cause of generalized epilepsy

The advances in molecular genetic methods has lead to the discovery of the genetic alterations that underlie the etiology of most diseases previously held to be idiopathic. Targeted genetic examination of a pediatric male patient showing a normal intellect, an extended area of skin hypopigmentation, and suffering from generalized epilepsy displaying a switch in epilepsy syndrome during the course of the disease towards a neurocutaneous syndrome was unsuccessful. Whole-exome sequencing identified a heterozygous missense mutation in a potassium chloride cotransporter gene, which together with the phenotype underscores the diagnosis of an epilepsy syndrome known in the literature as idiopathic generalized epilepsy type 14. Orv Hetil. 2019; 160(21): 835-838.