Urethral keratosis caused by vitamin A deficiency: two case reports.

BACKGROUND: Dysuria in children can have various etiologies, including ureteric stones, phimosis, congenital obstructive posterior urethral membrane, and neurogenic bladders. However, there have been no reports of vitamin A deficiency (VAD) causing dysuria due to urethral keratosis. Here, we report two cases of urethral keratosis caused by a VAD. CASE PRESENTATION: An 8-year-old boy (Patient 1) and a 6-year-old boy (Patient 2) presented with multiple episodes of dysuria and epididymitis. Both patients had intellectual disabilities and autism, which prevented voiding cystourethrography. Therefore, the patient was admitted for cystoscopy under general anesthesia. Cystoscopy revealed urethral diastolic dysfunction and a large amount of desquamated epithelium obstructing the urethra in both patients, causing urinary obstruction and dysuria. Catheterization was repeated; however, the symptoms recurred after catheter removal. Although the cause of recurrent urinary obstruction could not be initially identified, an ophthalmologist found Bitot's spots and suggested the possibility of a VAD. Serum vitamin A levels were extremely low in both patients, leading to the diagnosis of urethral keratosis due to VAD. VAD can be attributed to an unbalanced diet resulting from intellectual disabilities or autism. Vitamin A replacement therapy improved both the urethral symptoms and cystoscopic findings. CONCLUSION: Dysuria due to VAD is extremely rare, and urethral keratosis as a cause of dysuria is likely the first report of its kind worldwide. VAD may develop due to an unbalanced diet in patients with intellectual disabilities or autism. Therefore, it is essential to consider VAD as a potential cause of dysuria in patients with intellectual disabilities and autism.

Increase in pediatric complicated appendicitis during the COVID-19 pandemic: a multi-center retrospective study.

PURPOSE: An increase in the incidence of pediatric complicated appendicitis (CA) during the COVID-19 pandemic has been reported in many countries. We investigated how the pandemic has affected Japan. METHODS: We retrospectively reviewed children of ≤ 15 years old treated for acute appendicitis across 5 medical centers during the pandemic period (January to October in 2020), with the pre-pandemic period (January to October in 2017 to 2019) evaluated as a historical control. The incidence of CA and disease characteristics were then compared between the periods. RESULTS: The total number of patients was 55 in 2020 and 192 in 2017-2019. In all centers, the incidence of acute pediatric CA in the pandemic period significantly increased compared to the pre-pandemic period (18.2% vs. 32.7%, p = 0.02). On limiting our evaluation to the 3 institutions with reductions in patient numbers, the incidence of CA increased (16.3% vs. 37.9%, p = 0.01), and the duration of pre-operative symptoms was prolonged (1.3% vs. 1.7 days, p = 0.03). There were no significant differences in the age, sex, white blood cell count, C-reactive protein, or body temperature. No cases were diagnosed with SARS-CoV-2. CONCLUSIONS: The incidence of acute pediatric CA increased during the pandemic period. This may be related to an extended duration of symptoms due to individuals fearing contracting COVID-19 while visiting a hospital.

The outcomes of conservative management and the natural history of asymptomatic hydroceles in children.

PURPOSE: To elucidate the natural history of asymptomatic hydroceles (AHs) in children with conservative management and to discuss management strategies for AHs in children. METHODS: We retrospectively reviewed 113 children with AHs who were followed without immediate surgery. Patients were divided into four groups according to age at presentation; group 1: ≤ 1 month, group 2: 1-12 months, group 3: 12-24 months, and group 4: > 24 months. Ages at spontaneous resolution or surgery were reviewed. To assess the effect of AH on the testis, testicular size before and after conservative management was compared in 11 cases. RESULTS: In groups 1, 2, 3, and 4, spontaneous resolution occurred in 94.3%, 75.0%, 65.0%, and 33.3%, and the average time to resolution from presentation was 5, 5, 17, and 9 months, respectively. 41 patients underwent surgery at a mean age of 4.2 years. The testicular size did not differ significantly between before and after conservative management (14.4 vs. 14.5 mm, p = 0.483). CONCLUSION: About one-third of children over 2 years of age achieve spontaneous resolution. Hydroceles with conservative management may not affect the testicular size. Thus, initial conservative management before surgery is recommend for AHs in children, even over 2 years of age.

Management of inguinal hernia with prolapsed ovary in very low birthweight infants during neonatal intensive care unit hospitalisation.

AIM: Prolapsed ovary (PO) in an inguinal hernia (IH) may cause torsion and infarction; however, the management of IH with PO in very low birthweight (VLBW) infants during neonatal intensive care unit (NICU) hospitalisation remains inconsistent. METHODS: The medical records of 47 IHs in 30 VLBW infants during NICU hospitalisation between 2008 and 2017 were reviewed retrospectively. RESULTS: Of the 47 IHs, PO was diagnosed in 18 (38.3%). Post-natal age and body weight at diagnosis of IH with PO were 71 (44-172) days and 2120 (1305-2965) g, respectively. Seventeen IHs with PO underwent surgery for correction electively just before discharge from the NICU at the age and body weight of 94 (51-187) days and 2645 (2340-2945) g, respectively. Therefore, the time interval between diagnosis and surgery was 15 (7-90) days. There was no torsion of ovary before surgery and no postoperative complications, including apnoea and recurrence. CONCLUSIONS: The high incidence of IH with PO in VLBW infants during NICU hospitalisation was confirmed in this study. Under careful observation, elective IH repair just before discharge from the NICU could be one acceptable option for the management of IH with PO in VLBW infants during NICU hospitalisation to prevent torsion and infarction of ovary.

Thalidomide potentiates etoposide-induced apoptosis in murine neuroblastoma through suppression of NF-κB activation.

PURPOSE: Treatment for high-risk neuroblastoma is still challenging. The purpose of the present study was to determine whether thalidomide suppresses etoposide-induced NF-κB activation and thus potentiates apoptosis in murine neuroblastoma. METHODS: A murine neuroblastoma cell line, C1300, and A/J mice were used in this study. We evaluated NF-κB activation after using etoposide with or without thalidomide by quantitative analysis of NF-κB by ELISA and by Western blot analysis of IκB phosphorylation in vitro and in vivo. Induction of apoptosis was evaluated by Western blot analysis of the apoptotic signals caspase-3, 8, and 9 in vitro and by TUNEL assays in vivo. We also evaluated the efficacy of the combination of etoposide and thalidomide by assessing tumor growth and mouse survival in vivo. RESULTS: Etoposide activated NF-κB in C1300 cells. This activation was suppressed by thalidomide and IκB was re-upregulated. The apoptotic signals were enhanced by the combination of thalidomide and etoposide compared with etoposide alone in vitro, which was consistent with TUNEL assays. The combination of etoposide and thalidomide also slowed tumor growth and mouse survival. CONCLUSION: Thalidomide potentiates etoposide-induced apoptosis in murine neuroblastoma by suppressing NF-κB.

Clinical Insights Into Pediatric Solid Pseudopapillary Neoplasms of the Pancreas.

BACKGROUND: Solid pseudopapillary neoplasms (SPNs) of the pancreas are rare tumors with low malignant potential that usually occur in young girls and women. This study investigated the treatment experiences and outcomes after surgery for pediatric SPNs in our institutions as pediatric case series of solid SPNs are few, and long-term follow-up is also limited. METHODS: We retrospectively reviewed the outcomes of nine patients diagnosed with SPNs who underwent surgery in our three hospitals (The Jikei University Hospital, The Jikei University Kashiwa Hospital, and Kawaguchi Municipal Medical Center) between 2001 and 2023. RESULTS: All nine patients who underwent surgery were girls. Their ages ranged from 8 to 15 years (median: 10 years). The location of the tumor was at the pancreatic head, body, and tail in five, one, and three patients, respectively. Enucleation, pancreaticoduodenectomy, and laparoscopic distal pancreatectomy (LDP) were performed in four, two, and three patients, respectively. Regarding postoperative complications, a pancreatic fistula was detected in six patients, with three and three patients having grades A and B fistulas, respectively; two patients required percutaneous drainage, and one patient required endoscopic ultrasonography (EUS)-guided transgastric drainage. The follow-up period ranged from six to 261 months (median: 97 months). At the final follow-up, all nine patients were alive without recurrence. CONCLUSION: SPNs of the pancreas are incidentally diagnosed by diagnostic workups following trauma in children more frequently compared to adults. Additionally, the tumor resection by minimally invasive approaches, such as enucleation, or laparoscopic procedures results in a good prognosis in some cases.

The Comparison of Postoperative Umbilical Port Site-Related Complications Between Transumbilical and Periumbilical Incision After Laparoscopic Surgery in Children.

Introduction: No studies have directly compared postoperative umbilical port site (UPS)-related complications between transumbilical and periumbilical incisions (TUI and PUI) after laparoscopic surgery in children. Patients and Methods: We conducted a retrospective review of 324 children of ≤15 years of age who underwent laparoscopic repair (LR) for inguinal hernia, via either a TUI or PUI, between 2010 and 2020. UPS-related complications, such as wound infection and umbilical deformity, were compared between TUI and PUI. We also investigated the risk factors for the development of UPS-related complications after LR. Results: TUIs and PUIs were used for 228 and 96 children, respectively. The incidence rates of postoperative wound infection and umbilical deformity in the PUI group were higher in comparison to the TUI group; although not to a statistically significant extent (7.3% versus 5.3%, P = .451, 6.3% versus 4.8%, P = .593). One case of incisional hernia at the UPS was seen in the TUI group. In the univariate analysis, sex, age, weight for age Z-score, nutrition status, insertion and closure time, total operative time, American Society of Anesthesiologists score, blood loss, and comorbidities were not significantly associated with the development of UPS-related complications after LR. Conclusion: The incidence of UPS-related complications in TUI and PUI was not significantly different. Due to the low incidence of UPS-related complications, it was difficult to draw conclusions regarding contributing factors; however, meticulous care should be taken to avoid UPS-related complications when closing the UPS (Ethical approval No. 2019-24).

Risks and benefits of pediatric inguinal hernia repair: Conventional open repair vs laparoscopic percutaneous extraperitoneal closure.

INTRODUCTION: Considerable debate exists regarding the benefits of laparoscopic percutaneous extraperitoneal closure (LPEC) over conventional open repair (OR). We assessed the outcomes and feasibility of LPEC compared to OR for pediatric inguinal hernia (IH). METHODS: We retrospectively analyzed 570 children who underwent LPEC or OR. Parents decided the operative method after obtaining informed consent. Patient characteristics, operative time, complications and contralateral metachronous IH (CMIH) were compared between the groups. RESULTS: A total of 329 children underwent LPEC and 241 underwent OR. There was no significant difference in the incidence of recurrence or testicular ascent between the LPEC and OR groups (0.3% vs 0.4%, P = 0.825, 0.3% vs 0.8%, P = 0.391, respectively). No testicular atrophy was recognized in either group. One patient with postoperative chronic inguinal pain was recognized in each group. There was no surgical site infection (SSI) in the OR group; however, the LPEC group more frequently demonstrated umbilical port site (UPS)-related complications, such as incisional hernia, minor deformity, granuloma formation, cellulitis and superficial SSI. Ten (4.1%) developed CMIH in OR; in contrast, no case of CMIH was experienced after LPEC (P < 0.001). CONCLUSION: In conclusion, both LPEC and OR are feasible in the management of pediatric IH, because of their high success rates and low risk of complications. LPEC could be the superior procedure with respect to the prevention of CMIH. However, to maximize the merits of LPEC over OR, it is important to reduce UPS-related complications in LPEC. A longer follow-up is needed to assess male fertility in patients who receive LPEC.