Multidisciplinary management of disseminated Exophiala dermatitidis mycosis in an infant with mixed phenotype acute leukemia: a case report.

BACKGROUND: Exophiala dermatitidis is a dematiaceous fungus isolated from various environmental sources. Systemic E. dermatitidis infections can lead to fatal outcomes, and treatment has not yet been standardized. Although E. dermatitidis is also known to cause cutaneous infection, it has not been previously reported to appear as ecthyma gangrenosum (EG), an uncommon cutaneous lesion in neutropenic patients that is mainly caused by Pseudomonas aeruginosa. CASE PRESENTATION: A 2-month-old male infant with mixed-phenotype acute leukemia presented with prolonged fever unresponsive to antibacterial and antifungal agents during myelosuppression due to remission induction therapy. He also presented with skin lesions on the left wrist and left lower quadrant of the abdomen. The abdominal lesion gradually turned black and necrotic, which was consistent with the findings of the EG. E. dermatitidis was isolated from the blood, stool, wrist skin, and endotracheal aspirate. During hematopoietic recovery, consolidation in both lungs was evident. Multiagent antifungal treatment failed to eliminate E. dermatitidis from blood. In order to salvage the central venous catheter, ethanol lock therapy (ELT) was adopted, following which the blood culture became negative. The abdominal lesion that evolved as a necrotic mass connecting the small intestine and subcutaneous tissue adjacent to the skin was surgically resected. After these interventions, the general condition improved. CONCLUSION: Disseminated E. dermatitidis mycosis in the neutropenic infant was successfully managed with a multidisciplinary treatment consisting of multiagent antifungal treatment, ELT, and surgery.

The Role Of Surgical Treatment In The Multidisciplinary Therapy For Hepatoblastoma.

BACKGROUND/AIMS: The aim of this study was to evaluate the clinico-patholigical features and effects of multi-disciplinary therapy for pediatric patients with hepatoblastoma (HB). METHODOLOGY: Ten cases who underwent hepatectomy from 1996 to 2013 were studied. Our therapeutic protocol included neoadjuvant chemotherapy (NAC) + hepatectomy + adjuvant chemotherapy (AC). The regimens were CDDP+THP-ADR (n = 2), CITA (n = 1), PLADO (n = 5), and CDDP(n = 1). One case with 18-trisomy underwent only hepatectomy. Medical records were reviewed to evaluate characteristics, the effects of chemotherapy and prognosis. RESULTS: The median age at operation was 1.2 years (male: 7 and female: 3). PRETEXT classification was assigned to groups I (n = 2), II (n = 4), and III (n = 4). 15 years overall survival and disease-free survival rates were 100% and 80%, respectively. Two cases, who had post-operative lung metastasis, underwent chemotherapy with or without complete resection of lung tumors. No further recurrence was observed in these two cases. One case with 18 trisomy underwent right hepatectomy for PRETEXT II HB and is still doing well 67 months after hepatectomy. CONCLUSIONS: It was found that multidisciplinary therapy based on surgery, including complete resections of recurrent lesions, offers long-term survival, even for cases which develop recurrent metastatic tumors or cases complicated with 18 trisomy.

Criteria for Preserving Grossly Ischemic Torsed Testicles Using Indocyanine Green Fluorescence Imaging: A Single-Center Case Series.

Testicular torsion (TT) is a surgical emergency requiring early diagnosis and surgical intervention to avoid testicular loss. We report 3 pediatric TT cases with testicular salvage to visualize the blood flow by indocyanine green fluorescence imaging (ICG-FI) on the tunica albuginea of the testis. ICG-FI could not confirm blood flow in the testicular parenchyma; however, it could be detected in postoperative ultrasonography. Blood visualization of the tunica albuginea by ICG-FI may have potential criteria for testicular salvage. Long-term follow-up investigations after testicular salvage are needed to conclude the ICG-FI efficacy for TT.

Incidence of testicular microlithiasis after orchidopexy for cryptorchidism and its risk factors: A retrospective cohort study.

INTRODUCTION: Testicular microlithiasis (TML) is detected using scrotal ultrasound (US) and is characterized by hyperechoic non-shadowing foci 1-3 mm in diameter within the testicular parenchyma. Although a history of orchidopexy is a known risk factor for TML, and TML is a relatively common condition in postoperative cryptorchidism patients, no previous report has focused on risk factors for TML after orchidopexy. OBJECTIVE: The aims of this study were to evaluate the time-dependent incidence of TML after orchidopexy for cryptorchidism and to identify risk factors for TML. STUDY DESIGN: This study included patients who underwent primary orchidopexy for cryptorchidism between 2004 and 2018 and could be evaluated using scrotal US before and after the procedure. Patients with chromosomal abnormalities and those with preoperative TML were excluded. The Kaplan-Meier method was used to assess postoperative TML appearance. The Cox proportional hazard model was used to evaluate three potential risk factors for postoperative TML: higher testicular position (proximal to the external inguinal ring), delayed orchidopexy (after 18 months of corrected age), and bilateral cryptorchidism. RESULTS: A total of 214 testes from 163 patients were eligible for inclusion (operative age and follow-up time: 2.2 years ± 18 months and 6.3 years ± 46 months, respectively). TML was found in 14/163 patients (8.6%) and 17/214 testes (8.1%). No testicular tumors were discovered during follow-up. The time-dependent TML incidence was 6.0% at 5 years and 11.2% at 10 years after surgery (Summary figure). A higher testicular location proximal to the external inguinal ring was found to be an independent risk factor in the Cox proportional hazard model (hazard ratio 6.18, 95% confidence interval 1.37-27.9, Summary figure). DISCUSSION: Our findings show that the incidence of postoperative TML increases for approximately 10 years following orchidopexy and is associated with a higher testicular location proximal to the external inguinal ring at surgery. In the adult population, TML with a history of cryptorchidism is a known risk factor of testicular malignancy. Given that more than 20% of boys with a higher testicular position developed TML, US evaluation to detect TML during pubety is feasible for the patients with a history of orchidopexy. CONCLUSION: The incidence of postoperative TML in boys with cryptorchidism rises until puberty and is strongly associated with a higher testicular position at orchidopexy.

Medical English Education in Japan†:†Developing a Curriculum to Motivate Students by Providing Visualization Opportunities Using Near-peer Teaching.

Despite advancements in the pedagogy of medical education in various fields, Japan has no standardized medical English education. The U.S. Medical Licensing Examination (USMLE) Study Group of Tokushima is an extracurricular activity in which medical students and recent graduates meet every 1-2 months. The aim is to stimulate students' curiosity ; cultivate their initiative, self-efficacy, and English learning goals ; and motivate them to be self-regulated learners. Accordingly, we conducted near-peer teaching style lectures that focused on sharing medical English-related experiences, so students could have regular opportunities to visualize the benefits of learning medical English. Following the activities, we observed increased motivation and self-study among students, resulting in a high USMLE passing rate. Furthermore, five members started their training at American hospitals and pursued careers in English-speaking environments. Thus, near-peer teaching style leads to shared medical English-related experiences that help students to visualize English-related opportunities. This education style taught by similar generations aids in setting a specific goal by providing access to role models, cultivating their initiative and self-efficacy, motivating them to learn English, and producing positive outcomes.Modifying the curriculum to actively create opportunities for students to visualize themselves in an international environment can motivate them to continue learning English. J. Med. Invest. 69 : 332-334, August, 2022.

Hereditary paraganglioma presenting with atypical symptoms: Case report.

RATIONALE: Paraganglioma (PGL), an extra-adrenal pheochromocytoma, is a rare tumor, especially in children. While hypersecretion of catecholamines causes the classic triad of headaches, palpitations, and profuse sweating, prompt diagnosis is still challenging. PATIENT CONCERNS: For 7 months, an 8-year-old boy complained of polyuria and weight loss, followed by proteinuria and headache for 1 month prior to admission. He was admitted to our hospital due to an afebrile seizure. DIAGNOSIS: His blood pressure remained markedly elevated even after cessation of the convulsion. Magnetic resonance imaging of the brain revealed posterior reversible encephalopathy syndrome. Abdominal computed tomography showed a mass lesion encasing the left renal artery, measuring 41 mm in length along its major axis. The plasma and urine levels of normetanephrine were elevated. Additionally, iodine-123-metaiodobenzylguanidine scintigraphy showed an abnormal uptake in the abdominal mass with no evidence of metastasis. Based on these findings, we tentatively diagnosed him with PGL. INTERVENTION: Substantial alpha- and beta-blocking procedures were performed, followed by a tumor resection and an extended left nephrectomy on day 31 of hospitalization. Pathological findings confirmed the diagnosis of PGL. OUTCOME: The postoperative course was uneventful, and his blood pressure normalized without the use of antihypertensive agents. Genetic testing revealed a known SDHB germline mutation. The same mutation was also detected on his father and paternal grandfather without any history of hypertension or malignant tumor. LESSON: It remains challenging to diagnose pheochromocytoma/paraganglioma (PPGL) promptly because PPGL can present with a variety of symptoms. Preceding symptoms of the presented case might be caused by PGL. Although PPGL is a rare disease, especially in children, it should be considered in differential diagnosis when various unexplained symptoms persist.

Comparative analysis of robotic-assisted versus laparoscopic cholecystectomy in pediatric patients.

BACKGROUND: Despite increased utilization of robotic-assisted surgery in the pediatric population during the past decade, reports of comparative analysis between robotic surgery and laparoscopic surgery are lacking. Our aim was to evaluate outcomes between pediatric robotic-assisted cholecystectomy (RC) and laparoscopic cholecystectomy (LC). METHODS: A single institution retrospective analysis of 299 patients undergoing either RC or LC, between January 2015 and December 2018 was performed. Demographic data as well as clinical characteristics and related outcomes were abstracted and compared using univariate analysis. Related hospital costs were estimated using a charge to cost methodology. RESULTS: The median age of the cohort was 15.5 years (IQR 14.0-17.0); 76% females and 70% white, with 74% (n = 220) undergoing LC and 26% (n = 79) undergoing RC. The majority of RC were performed using single-site technique and RC proportion increased with time (10% in 2015 vs. 41% in 2018, p<0.001). The majority of RC were more commonly attributed to patients with nonacute indications for cholecystectomy compared to acute clinical indications (87% vs. 13%). Median operative time was 98 min vs. 79 min for RC and LC respectively (p<0.001). Median postoperative LOS was similar between groups (22 h). There were no significant differences in postoperative complication, in-hospital opioid utilization and 30-day readmissions. Average total hospital costs for RC were $15,519 compared to $11,197 for LC. CONCLUSIONS: Pediatric robotic-assisted cholecystectomy is feasible with similar outcomes compared to laparoscopic cholecystectomy. However, it is associated with longer operative times and higher costs. The single-site RC technique may provide a potential cosmetic benefit.

Recurrence after laparoscopic high ligation in adolescents: A multicenter international retrospective study of ten hospitals.

PURPOSE: Inguinal hernia repairs are among the most common operations performed by pediatric surgeons. Laparoscopic high ligation is a popular technique, but its recurrence rate in adolescents is unknown. We hypothesized that recurrence after laparoscopic high ligation in adolescents would be similar to open repair (1.8%-6.3%). METHODS: We evaluated adolescent patients (12-18 years old at the time of surgery) who underwent laparoscopic high ligation across eleven hospitals. At least six months postoperatively, they were contacted by telephone for follow-up. Variables analyzed included demographics, operative details, recurrence, and other complications. RESULTS: A total of 144 patients were enrolled. One hospital (n=9) had a recurrence rate of 44.4%, compared to 3.0% (4/135) for the other hospitals. By accounting for 50.0% of recurrences, it represented a statistical outlier and was excluded, leaving 135 patients for analysis. The median age was 14 years, and 63.7% were male. Recurrence with the excluded center was 5.6% (8/144). Use of absorbable suture (OR 42.67, CI 4.41-412.90, p<0.01) and braided suture (OR 12.10, CI 1.54-95.25, p=0.02) was weakly associated with recurrence. Recurrence was not significantly different from published results. CONCLUSION: Laparoscopic high ligation of adolescent inguinal hernias has a recurrence rate similar to open repair when performed by experienced surgeons. TYPE OF STUDY: Prognosis study (retrospective study) LEVEL OF EVIDENCE: Level II.

Treatment strategy for successful hepatic resection of icteric liver.

BACKGROUND: The treatment strategy for jaundiced patients with hilar cholangiocarcinoma (HC) is not well established. In this study, we evaluate the feasibility of our perioperative protocol for jaundiced patients with HC. METHODS: Twenty patients with HC who underwent hepatic resection at our institute were enrolled, and patients were divided into icteric(n=6) and normal(n=14) group. As a perioperative protocol, Oral administration of Inchinkoto(ICKT), steroid and nafamostat mesilate were introduced. The evaluation of functional future remnant liver(FRL) by asiaroscintigraphy, and postoperative outcomes were retrospectively compared. RESULTS: Indocyanine green dye retention rate at 15 minutes was higher, and LHL15 values was lower in icteric group. However, in the functional evaluation of FRL, which was the sum of GSA uptake of the future FRL, there was no significant difference of LHL15 values of the remnant liver functional reserve between the two groups. As results, according to the difference of liver function, serum AST level was not different between two groups. The number of patients with postoperative morbidity in the two groups was comparable. CONCLUSIONS: Even in HC patients with icteric liver, accurate assessment of liver functional reserve and effective perioperative treatment may attribute to successful hepatectomy and favorable post-operative outcomes. J. Med. Invest. 65:37-42, February, 2018.

Penetrative trace fossils from the late Ediacaran of Mongolia: early onset of the agronomic revolution.

The Cambrian radiation of complex animals includes a dramatic increase in the depth and intensity of bioturbation in seafloor sediment known as the 'agronomic revolution'. This bioturbation transition was coupled with a shift in dominant trace fossil style from horizontal surficial traces in the late Precambrian to vertically penetrative trace fossils in the Cambrian. Here we show the existence of the first vertically penetrative trace fossils from the latest Ediacaran: dense occurrences of the U-shaped trace fossil Arenicolites from late Precambrian marine carbonates of Western Mongolia. Their Ediacaran age is established through stable carbon isotope chemostratigraphy and their occurrence stratigraphically below the first appearance of the trace fossil Treptichnus pedum. These Arenicolites are large in diameter, penetrate down to at least 4 cm into the sediment, and were presumably formed by the activity of bilaterian animals. They are preserved commonly as paired circular openings on bedding planes with maximum diameters ranging up to almost 1 cm, and as U- and J-shaped tubes in vertical sections of beds. Discovery of these complex penetrative trace fossils demonstrates that the agronomic revolution started earlier than previously considered.

Manifestation of intraoperative anaphylactic shock along with latex allergy:a pediatric case report.

Natural rubber latex (NRL) allergy is one of the most important causes of severe anaphylaxis during medical intervention. We report a pediatric case of latex allergy with multiple surgical histories. A 12-year-old girl developed anaphylactic shock during the pyeloplasty for ureteropelvic junction restenosis. Latex gloves or medications used during the surgery were suspected to be the cause of anaphylactic shock. We diagnosed her latex allergy on the basis of the results that serum latex-specific IgE, skin prick tests of extract from NRL gloves and recombinant Hev b 6.02 solution were positive. Basophil activation test of NRL gloves was also positive, supporting the diagnosis of immediate allergic reactions caused by NRL. It was speculated that a history of multiple surgeries in infancy became a trigger of sensitization to latex in this patient. Reoperation after the diagnosis of NRL allergy was carried out in a latex-free environment and completed without any allergic symptoms. It would be necessary to perform the pre-screening of latex allergy to prevent the onset of latex allergy especially in the patients with multiple surgical histories. J. Med. Invest. 65:292-295, August, 2018.

Intrascrotal lipoblastoma: report of a case and the review of literature.

Intrascrotal lipoblastoma is a rare pediatric benign soft tissue neoplasm, and only 11 cases have been reported. The accurate preoperative diagnosis is difficult because of its rarelity and the similarity with the other soft tissue tumors. Among them, accurate preoperative diagnosis had been made in only one case. Thus, almost all of the cases had required inguinal mass excision (and orchidectomy in one case). In this paper, we discuss the accurate preoperative diagnosis of intrascrotal lipoblastoma and subsequent simple tumorectomy via minimal invasive scrotal skin incision, in 1-year-old boy. On physical examination, intrascrotal extra-testicular lobulated mass was palpated on the right scrotum. An ultrasonography revealed the well-circumscribed, iso-echoic, scant blood-flow, and lobulated tumors with each lobules of 1 to 4 cm in diameter, and the tumor located outside of the tunica vaginalis testis. The serum values of alpha-fetoprotein (AFP) and beta-human chorionic gonadotropin (b-hCG) were within normal limit. The preoperative diagnosis of intrascrotal lipoblastoma was made, and the mass was excised via minimal scrotal incision. The right testicle and epididymis were normal. The lesion consisted of the distinct two lobulated tumors, and microscopic examination confirmed the diagnosis of intrascrotal lipoblastoma. The postoperative course was uneventful without evidence of recurrence. A rare intrascrotal lipoblastoma is seldom made accurate preoperative diagnosis; however, the accurate preoperative suspicion of this tumor leads to the minimal invasive tumorectomy via scrotal skin incision and favorable postoperative recovery without recurrence.

The Kampo medicine "Daikenchuto (TU-100)" prevents bacterial translocation and hepatic fibrosis in a rat model of biliary atresia.

BACKGROUND: Biliary atresia is the most common cause of end-stage liver disease in children. It is known that bile duct ligation contributes to liver fibrosis via bacterial translocation (BT) and toll-like receptor 4 (TLR4) signaling of hepatic stellate cells (HSCs). We have reported previously that the traditional Japanese medicine, "Dai-kenchu-to (TU-100)," a form of "Kampo medicine" prevents BT in rats exposed to the stress of fasting. The aim of this study was to clarify the effect of TU-100 on a rat model of biliary atresia using bile duct ligation. METHODS: Bile duct ligation and subsequent daily oral administration of TU-100 was performed in 6-week-old rats. The rats were killed at 3, 7, or 14 days after bile duct ligation to evaluate the liver injury, occurrence of BT, and hepatic fibrosis. As an in vitro experiment, we isolated fresh HSCs from the rats undergoing bile duct ligation. After cell attachment, TU-100 and its 3 component herbs (eg, processed ginger, ginseng radix, and Japanese pepper) were added, and the expressions of Alpha actin2 (acta2), Alpha-1 type I collagen (colIa1), and tissue inhibitor of metalloproteinase 1 (timp1) were analyzed. RESULTS: In vivo experiments demonstrated that oral administration of TU-100 decreased liver injury and atrophy of intestinal mucosa BT, hepatic fibrosis, and hepatic expression of alpha smooth muscle actin (αSMA) and TLR4, compared with rats that underwent bile duct ligation only. In vitro experiments showed that administration of TU-100 or the component herbs inhibited the expressions of acta2, colIa1, and timp1 in the HSCs. CONCLUSION: TU-100 prevented BT, activation of HSCs, and subsequent hepatic fibrosis. TU-100 may prevent progression of hepatic fibrosis in children with biliary atresia and improve prognosis.

Laparoscopic resection of hepatoblastoma: report of a case.

Despite the increasing number of recent reports on laparoscopic resection of malignant liver tumors in adults, there have been few reports involving children. In this paper we discuss the laparoscopic resection of a hepatoblastoma in a 1-year-old boy. The CT scan revealed an exophytic hepatic tumor on S5 measuring 6 × 5 × 4 cm after preoperative chemotherapy. The operation time was 225 min with an estimated blood loss of about 38 mL; intraoperative transfusion was not required. Radiofrequency-assisted precoagulation was performed. The patient had an uncomplicated recovery and started postoperative chemotherapy on postoperative day 12. Our literature search revealed only five reported cases of laparoscopic resection of hepatoblastoma in which there was no postoperative complication or recurrence. Laparoscopic resection of a hepatoblastoma in a selected subgroup of patients can be safe and feasible.

Indigo carmine dye-assisted lymphatic-sparing laparoscopic Palomo varicocelectomy in children.

UNLABELLED: The ideal method for varicocelectomy in children remains controversial. We present our experience with dye-assisted lymphatic-sparing laparoscopic varicocelectomy (LSLV) in children, which overcomes the limitations of previously described techniques. MATERIALS AND METHODS: Five consecutive LSLVs were performed over a period of three years on children with a mean age of twelve years. The varicocele grade was three in one case and grade 2 in four cases, respectively. A left subdartos injection of 2 ml of Indigo carmine dye was done using a 25-gauge needle at ten minutes before an operation. A scrotal injection of lymphatic dye was utilized to spare at least one lymphatic and the remaining spermatic vessels were divided. RESULTS: Lymphatic-sparing was accomplished in all cases. No peri-operative complication was noted. We spared one lymphatic channel in one patient (20%) and two channels in four patients (80%). There were no cases of hydrocele or residual varicocele. CONCLUSION: Dye-assisted LSLV is easily accomplished with an excellent surgical outcome and sparing one or two lymphatics appears to be sufficient to avoid secondary hydrocele.

Iliopsoas abscess in an infant.

Iliopsoas abscess (IPA) is uncommon in childhood and very rare in the neonate and infant. We present a case of IPA after an umbilical granuloma in an infant. A baby girl with a birth weight of 2,970 g was born at thirty-seven weeks and two days gestation by Caesarean Section. On the fourteenth day after birth, her umbilicus was wet and developed the granulomatous formation. At two months of age, she presented with right leg and groin swelling with mild bluish discoloration and without fever. She had poor movement of her right leg and showed apparent discomfort. Her umbilicus was dry and there was no granulomatous material. Ultrasonography and computed tomography demonstrated an iliopsoas abscess in the right position. Therefore, an extraperitoneal surgical drainage was performed, aspirating yellowish pus. Culture of the purulent material revealed Staphylococcus aureus. Systemic antibiotic therapy was continued for ten days. After three days of drainage, full-range motion of the right leg was gained, and then after eleven days, CT findings comfirmed the disappearance of the iliopsoas abscess. IPA is extremely rare and it is difficult to diagnose. However, it should be included in the differential diagnosis of an infant with poor leg movement and swelling of the groin to the femur.