Moderate to severe osteogenesis imperfecta is associated with multiple fractures in childhood. There are no published data regarding the effects of third-generation bisphosphonates in these children. This randomized study investigated which of three different doses of risedronate was most effective in reducing fracture incidence. We randomly assigned 53 children with moderate to severe osteogenesis imperfecta to receive 0.2, 1, or 2 mg/kg per week of risedronate. We assessed safety, fracture incidence, and bone measurement outcomes at 3, 6, 12, 18, and 24 months. At 24 months, 69% of children assigned 0.2 mg/kg per week had had new fractures compared with 44% receiving 1 mg/kg per week and 75% receiving 2 mg/kg per week. Poisson regression with age and prior fracture as covariates showed that there was no difference in incident nonvertebral fracture between groups. Fracture rate diminished in each group during the trial compared with previous the 2 years ( p ¼ .005). Lumbar spine bone mineral density increased significantly ( p ¼ .009) only in the 2 mg/kg per week group. Long bone bowing deformities reduced more in children receiving 1 or 2 mg/kg per week of risedronate [odds ratio (OR) 0.67, 95% confidence interval (CI) 0.48-0.93 per unit increase in risedronate dose, p ¼ .015]. There were no serious adverse events. Bone mass increased and bowing deformities reduced with increasing risedronate dose. Children suffered fewer fractures irrespective of risedronate dose. The most appropriate dose of risedronate for children with moderate to severe osteogenesis imperfecta in this study was 2 mg/kg per week. ß
Our review of the records at 6 centers revealed a combined 0.17% incidence of rectal injury. This compares favorably to the incidence in modern open and laparoscopic radical prostatectomy series. No preoperative, intraoperative or pathological differences correlated with injury. Cases in which rectal injury was identified intraoperatively required fewer surgical repeat interventions but ultimately each group had acceptable long-term urinary and bowel function results.
Purpose Children with von Hippel-Lindau syndrome are at an increased risk for developing bilateral pheochromocytomas. In an effort to illustrate the advantage of partial adrenalectomy (PA) over total adrenalectomy in children with VHL, we report the largest single series on PA for pediatric VHL patients, demonstrating a balance between tumor removal and preservation of adrenocortical function. Methods From 1994 to 2011, a prospectively maintained database was reviewed to evaluate 10 pediatric patients with hereditary pheochromocytoma for PA. Surgery was performed if there was clinical evidence of pheochromocytoma and normal adrenocortical tissue was evident on preoperative imaging and/or intraoperative ultrasonography. Perioperative data were collected and patients were followed for postoperative steroid use and tumor recurrence. Results Ten pediatric patients with a diagnosis of VHL underwent 18 successful partial adrenalectomies (4 open, 14 laparoscopic). The median tumor size removed was 2.6 centimeters (range 1.2–6.5). Over a median follow up of 7.2 years (range 2.6–15.8) additional tumors in the ipsilateral adrenal gland were found in two patients. One patient underwent completion adrenalectomy and one underwent a salvage PA with resection of the ipsilateral lesion. One patient required short term steroid replacement therapy. At last follow up, 7 patients had no radiographic or laboratory evidence of pheochromocytoma. Conclusion At our institution, partial adrenalectomy is the preferred form of management for pheochromocytoma in the (VHL) pediatric population. This surgical approach allows for removal of tumor while preserving adrenocortical function and minimizing the side effects of long term steroid replacement on puberty and quality of life.
Background: Miniaturization of endoscopic instruments in percutaneous nephrolithotomy (PCNL) allowed less invasive procedures with low complication rates, especially in children. This study was conducted to evaluate the safety and efficacy of ultrasonography-guided (USG) versus fluoroscopy-guided (FG) mini-PCNL in children.Materials and methods: This is a retrospective comparative study conducted from June 2015 to June 2020. The sample included 70 children (35 pateints underwent USG mini-PCNL and 35 pateints underwent FG mini-PCNL). They were compared mainly by the patients’ demographic characteristics, procedural information, and post-treatment outcomes. In the USG mini-PCNL group, puncturing was performed using a 3.5 MHz US probe, whereas fluoroscopy was utilized in the FG mini- PCNL group. Results: Both groups were comparable in terms of gender, previous history of failed ESWL, and hydronephrosis grade. The mean stone burden was 15.94 ± 3.69 mm and 19.20 ± 7.41 mm in USG and FG groups, respectively (p = 0.024). The stonefree rate (SFR) was 97.1% in the USG group and 94.3% in the FG group, which was not statistically significant (p = 0.16). Mean operative time in the USG group and FG group was 69.00 ± 13.33 minutes and 63.48 ± 16.90 minutes, respectively. Four (11.4%) patients in the FG group required blood transfusions to restore the hemodynamic state (p = 0.039). Fever was detected in 4 (11.4%) patients in the USG group and 15 (31.4%) patients in the FG group (p = 0.041). Conclusions: In children, mini PCNL under USG is safe and as effective as fluoroscopy.
ObjectiveTo evaluate the accuracy of ultrasonography (US) in measuring the urinary tract stone using non-contrast computed tomography (NCCT) as the standard reference.Patients and methodsA total of 184 patients suspected with urolithiasis who had undergone NCCT and US radiologic investigation from 2015 to 2017 were enrolled in this study. The sensitivity, specificity, and stone size measured in US were validated by NCCT. Data of the stone size in US were classified into four groups (0–3.5, 3.6–5, 5.1–10, >10 mm) and then compared with NCCT data.ResultsIn 184 patients, NCCT detected 276 (97.2%) stones, while US could identify 213 (75.5%) stones. Overall sensitivity and specificity of US were 75.4% and 16.7%, respectively. Detection rate of mid and distal ureteral stone was lower than that at other locations. The detection rate increased with the stone size. About 73% concordance was obtained for the stone size measured by US and NCCT (Pearson’s correlation coefficient was 0.841). Factors such as the stone size, amount of hydronephrosis, and weight affected the detection rate of the urinary tract stone using US (P<0.001, P=0.02, and P=0.01, respectively).ConclusionThe stone size obtained by US was almost the same as that detected by NCCT; however, US is a limited imaging modality in detecting urinary tract stone, especially when used by an inexperienced radiologist, and in the case of smaller stone size, increased weight, and low grade of hydronephrosis.
Purpose: Circumcision is one of the most prevalently performed surgeries worldwide. It is a common non-therapeutic procedure, which, based on the cultural and social background of people, could be performed by individuals at different levels of skill ranging from trained medical professionals to non-specialists. Therefore, this surgery could cause complications of varying frequencies and types. For example, penile damage during the circumcision procedure is a severe and rare complication leaving patients with morbidity and lifelong consequences. Here, we reported two cases of penile injury during a ritual circumcision. Patients and Methods: This study was approved by the ethics committee of Ibb University of Medical Sciences. Case 1: A 6-month-old child was brought to hospital by parents with complaints of purulent discharge around the penis after ritual circumcision performed 3 days ago with complete skin loss in the whole part of the penis extended to the scrotum and suprapubic area. Case 2: A 2-month-old child was brought to hospital with complaints of penile discharge and gangrene after ritual circumcision performed 10 days ago. Results: Case 1: He was treated with daily debridement and wound irrigation followed by a surgical procedure to bury the penis in the scrotum for future reconstructive surgery. Case 2: A suprapubic cystostomy was inserted into the bladder. Then, daily debridement and wound irrigation was performed for the child. To treat penile gangrene, two options were available. The first option was to bury the penis in the scrotum and do reconstructive surgery later. The second option was to change the child's anatomical sex from male to female. Conclusion: We suggest that every child, before a circumcision procedure, should be evaluated by a medically trained professional. This procedure should also be carried out in aseptic conditions. Additionally, the circumcision procedure needs to be performed by an experienced medical professional.
Complications after RALP are most commonly minor, requiring expectant or medical management only, even during the initiation of a RALP program. The complication rate improved significantly during the study period.
Background Etiological factors involved in chronic prostatitis (CP) type IIIb and chronic pelvic pain are not sufficiently understood; however, the nervous system has a significant role in the generation and maintenance of chronic pelvic pain. This study was designed to evaluate the sympathetic skin response (SSR) in men with CP type IIIb compared to normal population. Patients and Methods For two years, about 14 patients suffering from CP type IIIb according to NIH-CPSI and 26 healthy control men were enrolled in this study. SSR was performed in all the subjects with a standard method. Bilateral palmar and plantar latency and amplitude of SSR were recorded in response to the median and tibial nerve electrical stimulations. SSR is considered abnormal when the latency is prolonged, and the amplitude reduced. Results SSR latency in the left and right median nerve was significantly prolonged in the patient with CP type IIIb group compared to the control group (p=0.039 and 0.006, respectively). Additionally, the amplitude was reduced in patients with CP type IIIb group compared to the control group in the right tibial nerve (p=0.017). Conclusion Sympathetic skin response may be a helpful diagnostic test for men with chronic prostatitis type IIIb. However, this observation needs to be validated in a large sample cohort study with long-term follow-up.
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