Transient mutism has ben known as a rare complication following a posterior fossa approach to cerebellar tumors, although its pathophysiology remains unclear. Cerebellar mutism may be defined as a transient mutism occurring after a posterior fossa tumor approach in children with unimpaired consciousness, unimpaired symbolic functions, no detectable deficit of cranial nerves or peripheral organs of speech, and no lesions of long pathways in the course of the cranial nerves at the level of the brain stem. Forty two cases in children, and only two cases in adults have been found in the literature. We report a case of transient mutism with oropharyngeal apraxia after total excision of 4th ventricle choroids plexus papilloma in a 6-year-old girl. Postoperatively, the patient became mute and refused food intake without disturbance of consciousness, or lower cranial nerve function. Computed tomography(CT) and magnetic resonance image(MRI) demonstrated no abnormal findings except the surgical lesion in the posterior fossa. The mutism lasted for 3 weeks, but she gradually became fluent. The mechanism of "cerebellar mutism" is briefly discussed with literature review.
Adult ; Apraxias ; Brain Stem ; Cerebellar Neoplasms ; Child* ; Choroid Plexus* ; Choroid* ; Consciousness ; Cranial Nerves ; Eating ; Female* ; Humans ; Infratentorial Neoplasms ; Mutism* ; Papilloma ; Papilloma, Choroid Plexus*

To systematically review relevant literature on efficacy and safety of immune checkpoint inhibitors (ICIs) in patients with advanced and metastatic urothelial cell cancer (UCC), renal cell cancer (RCC), and prostate cancer. In platinum pretreated UCC, efficacy of pembrolizumab was superior to chemotherapy, with longer median overall survival (OS; 10.3 months vs. 7.4 months), a higher objective response rate (ORR; 21.1% vs. 11.4%, p=0.001), and a lower adverse event rate (60.9% vs. 90.2%). Three randomized controlled trials (RCTs) assessed the safety and efficacy of nivolumab in advanced RCC. The median OS (25.0 months vs. 19.6 months) and the ORR (25% vs. 5%) were higher in patients treated with nivolumab compared with second-line everolimus. In patients with metastatic castration-resistant prostate cancer, 2 RCTs were identified, which did not show significant benefits for ipilimumab over placebo. In UCC and RCC, there was no conclusive association between programmed cell death receptor ligand 1 (PD-L1) expression in tumor tissue and clinical outcome during pembrolizumab and nivolumab treatment, respectively. Therefore, in metastatic UCC and RCC, pembrolizumab and nivolumab have superior efficacy and safety to second-line chemotherapy and everolimus, respectively. No beneficial effect of ipilimumab was observed in prostate cancer patients. PD-L1 expression status is currently not suitable as a predictive marker for treatment outcome.
Carcinoma, Renal Cell ; Cell Death ; Drug Therapy ; Everolimus ; Humans ; Immunotherapy ; Platinum ; Prostatic Neoplasms ; Treatment Outcome ; Urologic Neoplasms

Sheehans syndrome is a known complication of pregnancy, It was described as a syndrome of hypopituitarysm due to acute ischemic necrosis of the anterior pituitary gland secondary to severe postpartum bleeding and shock. The neurophysis is usually preserved. But it can be involved in severe cases manifesting as diabetes insipidus. Because of its rare coexistence with Sheehans syndrome, diabetes insipidus is seldom recognized as a potential postpartum complication. The report describes a patient who developed Sheehans syndrome and diabetes insipidus immediately following delivery. Diabetes insipidus resolved spontaneously after 15 months, while panhypopituitarysm is persistent.
Diabetes Insipidus ; Hemorrhage ; Humans ; Hypopituitarism ; Necrosis ; Pituitary Gland, Anterior ; Postpartum Hemorrhage ; Postpartum Period ; Pregnancy ; Shock

PURPOSE: Febrile convulsion (FC) is the most common type of seizure disorder during childhood. The most frequent sequale after the first FC is the recurrence of another FC. A number of risk factors associated with the recurrence have been identified. We evaluated the factors associated with the recurrence of FC. METHOD: The recurrence rates of FC by the predictive factors of recurrence were retrospectively evaluated in a cohort of 214 Korean children who were admitted to the Pediatric Depatment of the Yonsei University Wonju College of Medicine, from January 1996 to December 1997. RESULT: The overall recurrence rate of FC was 56.1% and the cumulative rates of the recurrent cases by intervals between the initial onset and recurrence were 52.5% by 6 months, 82.5% by 12 months, and 89.2% by 24 months. When the onset of the first FC occurred less than 12 months of age, the recurrence rate (66.1%) was significantly higher than that of the other age groups of the onset (p<0.05). There were no differences in the recurrence rate by the other predictive factors-durations of the initial FC, seizure type of the initial FC, the recurrence during the first 24 hours after the initial FC, and the family histories of FC or epilepsy. CONCLUSION: We can conclude that the recurrence of FC statistically correlated with the age at onset, especially less than 12 months old. And we recommend that it may be necessary to prophylactically treat recurrence of FC if the first FC occurred at less than 12 months of age.
Child ; Cohort Studies ; Epilepsy ; Gangwon-do ; Humans ; Infant ; Recurrence ; Retrospective Studies ; Risk Factors* ; Seizures ; Seizures, Febrile*

A primary malignant melanoma occurring within the male urethra is a very rare disease, which usually shows highly malignant potential. Due to the low incidence, the clinical features of this disease remain unclear, and the mode of treatment controversial. Herein, we report a case of a malignant melanoma of the male urethra in a 77 year old man, who was managed by transurethral resection, and has remained uneventful for a 6 month follow-up period.
Aged ; Follow-Up Studies ; Humans ; Incidence ; Male* ; Melanoma* ; Rare Diseases ; Urethra*

Transverse vaginal septum, especially completely imperforate condition, is a rare congenital anomaly of female genital tract. The patient was 26 years old and visited for primary amenorrhea and low abdominal pain. Secondary sexual characteristics were all normal, but the depth of the vagina was only about 1cm. Hematometra was suspected and two large cystic structures were detected in the pelvis on ultrasonography. Intravenous pyelography and other laboratory results were normal. Vaginoplasty was needed to drain out the hematometra. We tried a new surgical technique using radial incisions of the closed vaginal end. To prevent postoperative contracture, we used a sterile pen case. and educated the patient to dilatate the vagina with that, daily. Here, we report a case of complete imperforate transverse septum with reviews of literatures.
Abdominal Pain ; Adult ; Amenorrhea ; Contracture ; Female ; Hematometra ; Humans ; Pelvis ; Ultrasonography ; Urography ; Vagina

Although intravesical instillation of Mycobacterium bovis bacillus Calmette-Guérin (BCG) is the most successful cancer immunotherapy for superficial bladder cancer, the serious side effects are frequently arisen by using live mycobacteria. To allow less toxic and more potent immunotherapeutic agents following intravesical BCG treatment for superficial bladder cancer, noninfectious immunotherapeutic drug instead of live BCG would be highly desirable. Recently, immune-enhancing adjuvants are considered an effective vaccine immunotherapy for cancer, providing enhanced antitumor effects and boosted immunity. The BCG-cell wall skeleton (BCG-CWS), the main immune active center of BCG, is a potent candidate as a noninfectious immunotherapeutic drug instead of live BCG against bladder cancer. However, the most limited application for anticancer therapy, it is difficult to formulate a water-soluble BCG-CWS due to the aggregation of BCG-CWS in both aqueous and nonaqueous solvents. To overcome the insolubility and improve the internalization of BCG-CWS into bladder cancer cells, it should be developed the lipid nanoparticulation of BCG-CWS, resulting in improved dispensability, stability, and small size. In addition, powerful technology of delivery systems should be applied to enhance the internalization of BCG-CWS, such as encapsulated into lipid nanoparticles using novel packaging methods. Here, we describe the progress in research on effects of BCG-CWS for cancer immunotherapy, development of lipid-based solvent, and packaging method using nanoparticles with drug delivery system.
Administration, Intravesical ; Bacillus ; Cell Wall Skeleton ; Drug Delivery Systems ; Immunotherapy ; Methods ; Mycobacterium bovis ; Nanoparticles ; Product Packaging ; Skeleton ; Solvents ; Urinary Bladder Neoplasms ; Urinary Bladder

Purpose: Although Mycobacterium bovis Bacillus Calmette-Guérin (BCG) is the most widely used bladder cancer immunotherapy, innate immune responses involving antimicrobial peptides (AMPs) cause BCG failure. Here, we developed genetically modified recombinant BCG (rBCG) strains which escape AMPs and evaluate the efficacy and effects of rBCG. Materials and Methods: We constructed rBCG strains expressing Streptococcal inhibitor of complement (Sic), which confers resistance to human α-defensin-1 and cathelicidin, and d-alanyl carrier protein ligase (dltA), which confers resistance to cationic AMPs. Sic and dltA were separately cloned into the pMV306 plasmid and introduced into BCG via electroporation. The efficacy of the Sic and dltA gene electroporation into BCG was evaluated by quantitative real-time polymerase chain reaction (qRT-PCR). The internalization rates and anticancer effects of the rBCG strains containing Sic (rBCG-Sic) and dltA (rBCG-dltA) was evaluated by the orthotopic bladder cancer mouse model. Results: The cycle quantification (Cq) values of rBCG-Sic (y=-4.8823x+13.645, R2=0.9996) and rBCG-dltA (y=-5.438x+11.641, R2=0.9995) were inverse correlations to the amount of Sic and dltA genes dose dependently. The mean introduction proportions of Sic and dltA genes into BCG by electroporation were 22.2%, 27.5% and showed constant efficacy. In the orthotopic bladder cancer mouse model, the relative internalization number of rBCG-Sic, and rBCG-dltA into bladder cell in mouse bladder were higher than that of BCG and the tumor volume at rBCG-Sic were lower than at BCG and rBCG-dltA at 11, 14 and 18 days. Conclusions Our results showed that constructed rBCG-Sic and rBCG-dltA by electroporation and the rBCG-Sic and rBCG-dltA can effectively escape BCG-stimulated AMPs, and significantly improved immunotherapeutic tools to treat bladder cancer in orthotopic bladder cancer mouse model.

Although intestinal bleeding is known to occur in amyloidosis, it is rare as a presenting symptom or sole manifestation of the disease. We experienced a case of intestinal hemor-rhage in a 64-year old female patient with primary amyloidosis, kappa type. Antral muco-sal erosions were discovered and one shallow healing ulcer at the angle. Colonoscopy revealed multiple purplish nodules in the sigmoid colon and descending colon, as well as a large shallow ulcer with blood clots in the sigmoid colon which was suspected to be the intestinal hemorrhage site. Endoscopic biopsy established amyloidosis. Polarizing microscopy after Congo red staining and immunohistochemical staining identified primary amyloid, kappa type. A case of intestinal hemorrhage in patients with primary amyloidosis is presented and the literature is reviewed.
Amyloid ; Amyloidosis* ; Biopsy ; Colon, Descending ; Colon, Sigmoid ; Colonoscopy ; Congo Red ; Female ; Hemorrhage* ; Humans ; Microscopy ; Middle Aged ; Ulcer

Alagille syndrome is characterized by chronic cholestasis, posterior embryotoxon, skeletal abnormalities, cardiovascular abnormalities, and a typical face with prominent forehead and pointed chin. Its histological feature includes paucity of interlobular bile ducts. We experienced a 49-day-old female infant presenting with frequent upper respiratory tract infection and persistent jaundice. She had a typical face and chronic cholestasis. Echocardiograms revealed peripheral pulmonary stenosis. The histological examination of liver revealed paucity of interlobular bile ducts.
Alagille Syndrome* ; Cardiovascular Abnormalities ; Chin ; Cholestasis ; Female ; Forehead ; Humans ; Infant ; Jaundice ; Liver ; Pulmonary Valve Stenosis ; Respiratory Tract Infections