Hemophagocytic lymphohistiocytosis (HLH) is characterized by macrophage and cytotoxic lymphocyte hyperactivation, fever, pancytopenia, liver dysfunction, and abnormal coagulation. However, no specific treatments have been established for HLH caused by immune checkpoint inhibitors.
Case Presentation
A 63-year-old male with clear cell renal carcinoma was treated with pembrolizumab and lenvatinib. Fifteen days later, he developed pancytopenia, liver and renal impairments, hypofibrinogenemia, hypertriglyceridemia, and elevated ferritin levels. Subsequently, he was admitted to the ICU for respiratory and circulatory instabilities. The patient was diagnosed with HLH and treated with high-dose corticosteroids and mycophenolate mofetil. Pancytopenia persisted and required massive blood transfusions. Cytomegalovirus infection was found to be the cause, and pancytopenia improved with ganciclovir. The patient was discharged from the ICU after 21 days.
Conclusion
We present the case of a patient who developed HLH as an immune-related adverse event along with a secondary cytomegalovirus infection, resulting in prolonged pancytopenia.
{"title":"A Case of Hemophagocytic Lymphohistiocytosis During Immune Checkpoint Inhibitor Treatment for Metastatic Renal Cell Carcinoma, Complicated by Pancytopenia Attributed to Cytomegalovirus Infection","authors":"Tomoko Honda, Hirohito Naito, Yu Osaki, Yoichiro Tohi, Yuki Matsuoka, Takuma Kato, Homare Okazoe, Rikiya Taoka, Nobufumi Ueda, Mikio Sugimoto","doi":"10.1002/iju5.70058","DOIUrl":"https://doi.org/10.1002/iju5.70058","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>Hemophagocytic lymphohistiocytosis (HLH) is characterized by macrophage and cytotoxic lymphocyte hyperactivation, fever, pancytopenia, liver dysfunction, and abnormal coagulation. However, no specific treatments have been established for HLH caused by immune checkpoint inhibitors.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case Presentation</h3>\u0000 \u0000 <p>A 63-year-old male with clear cell renal carcinoma was treated with pembrolizumab and lenvatinib. Fifteen days later, he developed pancytopenia, liver and renal impairments, hypofibrinogenemia, hypertriglyceridemia, and elevated ferritin levels. Subsequently, he was admitted to the ICU for respiratory and circulatory instabilities. The patient was diagnosed with HLH and treated with high-dose corticosteroids and mycophenolate mofetil. Pancytopenia persisted and required massive blood transfusions. Cytomegalovirus infection was found to be the cause, and pancytopenia improved with ganciclovir. The patient was discharged from the ICU after 21 days.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>We present the case of a patient who developed HLH as an immune-related adverse event along with a secondary cytomegalovirus infection, resulting in prolonged pancytopenia.</p>\u0000 </section>\u0000 </div>","PeriodicalId":52909,"journal":{"name":"IJU Case Reports","volume":"8 4","pages":"423-426"},"PeriodicalIF":0.0,"publicationDate":"2025-06-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/iju5.70058","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144524686","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Spermatogenic dysfunction is a side effect of valganciclovir, which is commonly used to treat cytomegalovirus infections. Here, we report the case of a reproductive man who underwent kidney transplantation and was diagnosed with azoospermia after valganciclovir treatment for cytomegalovirus infection.
Case Presentation
A 30-year-old man underwent an ABO-compatible living-donor kidney transplantation. Two months after the kidney transplant, the patient was diagnosed with cytomegalovirus infection and gastritis. Therefore, valganciclovir treatment was initiated. Two months after completion of valganciclovir treatment, the patient was diagnosed with azoospermia. Azoospermia induced by valganciclovir was suspected, and careful monitoring was performed. Nine months after azoospermia diagnosis, the sperm concentration improved to normal range (43.3 × 106/mL). Subsequently, the patient's wife conceived.
Conclusion
During valganciclovir treatment, careful monitoring and adequate informed consent are needed to support patients of reproductive age.
{"title":"Transient Azoospermia Induced by Valganciclovir Treatment for Cytomegalovirus Infection in a Reproductive Male After Kidney Transplant: A Case Report","authors":"Kosuke Mieda, Shunta Hori, Mitsuru Tomizawa, Kuniaki Inoue, Tatsuo Yoneda, Yuji Nitta, Yasushi Nakai, Makito Miyake, Nobumichi Tanaka, Kiyohide Fujimoto","doi":"10.1002/iju5.70060","DOIUrl":"https://doi.org/10.1002/iju5.70060","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>Spermatogenic dysfunction is a side effect of valganciclovir, which is commonly used to treat cytomegalovirus infections. Here, we report the case of a reproductive man who underwent kidney transplantation and was diagnosed with azoospermia after valganciclovir treatment for cytomegalovirus infection.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case Presentation</h3>\u0000 \u0000 <p>A 30-year-old man underwent an ABO-compatible living-donor kidney transplantation. Two months after the kidney transplant, the patient was diagnosed with cytomegalovirus infection and gastritis. Therefore, valganciclovir treatment was initiated. Two months after completion of valganciclovir treatment, the patient was diagnosed with azoospermia. Azoospermia induced by valganciclovir was suspected, and careful monitoring was performed. Nine months after azoospermia diagnosis, the sperm concentration improved to normal range (43.3 × 10<sup>6</sup>/mL). Subsequently, the patient's wife conceived.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>During valganciclovir treatment, careful monitoring and adequate informed consent are needed to support patients of reproductive age.</p>\u0000 </section>\u0000 </div>","PeriodicalId":52909,"journal":{"name":"IJU Case Reports","volume":"8 4","pages":"431-434"},"PeriodicalIF":0.0,"publicationDate":"2025-06-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/iju5.70060","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144524685","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Posterior reversible leukoencephalopathy syndrome (PRES) is a rare but serious complication in kidney transplant recipients, often triggered by calcineurin inhibitors (CNIs) and infections.
Case Presentation
A 52-year-old woman with end-stage kidney disease underwent cadaveric renal transplantation. Two months post-transplant, she presented with headaches, visual disturbances, hypertension, and altered consciousness. Cranial MRI confirmed PRES. After conversion from tacrolimus to cyclosporine and antihypertensive therapy, symptoms improved. However, the patient developed disseminated varicella-zoster virus infection, resulting in meningitis. Treatment with acyclovir and reduction of immunosuppression led to full recovery without recurrence.
Conclusion
This case highlights the importance of recognizing PRES and its triggers, including infections and CNIs, in kidney transplant recipients. Early diagnosis and appropriate management are crucial for preventing severe outcomes.
{"title":"Posterior Reversible Leukoencephalopathy Syndrome and Disseminated Varicella-Zoster Virus Infection After Kidney Transplantation","authors":"Kenji Tsutsui, Shigeaki Nakazawa, Makoto Kinoshita, Yoko Higa, Soichi Matsumura, Shota Fukae, Ryo Tanaka, Norichika Ueda, Yoichi Kakuta, Norio Nonomura","doi":"10.1002/iju5.70030","DOIUrl":"https://doi.org/10.1002/iju5.70030","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>Posterior reversible leukoencephalopathy syndrome (PRES) is a rare but serious complication in kidney transplant recipients, often triggered by calcineurin inhibitors (CNIs) and infections.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case Presentation</h3>\u0000 \u0000 <p>A 52-year-old woman with end-stage kidney disease underwent cadaveric renal transplantation. Two months post-transplant, she presented with headaches, visual disturbances, hypertension, and altered consciousness. Cranial MRI confirmed PRES. After conversion from tacrolimus to cyclosporine and antihypertensive therapy, symptoms improved. However, the patient developed disseminated varicella-zoster virus infection, resulting in meningitis. Treatment with acyclovir and reduction of immunosuppression led to full recovery without recurrence.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>This case highlights the importance of recognizing PRES and its triggers, including infections and CNIs, in kidney transplant recipients. Early diagnosis and appropriate management are crucial for preventing severe outcomes.</p>\u0000 </section>\u0000 </div>","PeriodicalId":52909,"journal":{"name":"IJU Case Reports","volume":"8 4","pages":"330-333"},"PeriodicalIF":0.0,"publicationDate":"2025-06-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/iju5.70030","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144524743","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Akari Hiraguri, Yuichi Sato, Junya Hata, Yusuke Kirihana, Akihisa Hasegawa, Satoru Meguro, Kanako Matsuoka, Seiji Hoshi, Souichiro Ogawa, Yoshiyuki Kojima
Introduction
A case of bilateral high intra-abdominal testes successfully treated with multistage Fowler–Stephens orchiopexy is reported.
Case Presentation
A 6-month-old boy with bilateral nonpalpable testes was diagnosed with a left intra-abdominal testis located immediately caudal to the spleen on magnetic resonance imaging and laparoscopy. At the age of 4 years, diffusion-weighted magnetic resonance imaging detected a structure immediately caudal to the liver, which was suspected to be the right testis. With a diagnosis of bilateral intra-abdominal testes, one-stage Fowler–Stephens orchiopexy was performed on the right testis at 5 years of age. After confirming its development, two-stage Fowler–Stephens orchiopexy was performed on the left testis at 10 years of age.
Conclusion
After confirming the development of the right testis that underwent one-stage Fowler–Stephens orchiopexy, two-stage Fowler–Stephens orchiopexy was completed on the left testis, resulting in the successful preservation of both testes and normal sexual development.
{"title":"Bilateral High Intra-Abdominal Testes Successfully Treated With Multistage Fowler–Stephens Orchiopexy to Preserve Testicular Function","authors":"Akari Hiraguri, Yuichi Sato, Junya Hata, Yusuke Kirihana, Akihisa Hasegawa, Satoru Meguro, Kanako Matsuoka, Seiji Hoshi, Souichiro Ogawa, Yoshiyuki Kojima","doi":"10.1002/iju5.70059","DOIUrl":"https://doi.org/10.1002/iju5.70059","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>A case of bilateral high intra-abdominal testes successfully treated with multistage Fowler–Stephens orchiopexy is reported.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case Presentation</h3>\u0000 \u0000 <p>A 6-month-old boy with bilateral nonpalpable testes was diagnosed with a left intra-abdominal testis located immediately caudal to the spleen on magnetic resonance imaging and laparoscopy. At the age of 4 years, diffusion-weighted magnetic resonance imaging detected a structure immediately caudal to the liver, which was suspected to be the right testis. With a diagnosis of bilateral intra-abdominal testes, one-stage Fowler–Stephens orchiopexy was performed on the right testis at 5 years of age. After confirming its development, two-stage Fowler–Stephens orchiopexy was performed on the left testis at 10 years of age.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>After confirming the development of the right testis that underwent one-stage Fowler–Stephens orchiopexy, two-stage Fowler–Stephens orchiopexy was completed on the left testis, resulting in the successful preservation of both testes and normal sexual development.</p>\u0000 </section>\u0000 </div>","PeriodicalId":52909,"journal":{"name":"IJU Case Reports","volume":"8 4","pages":"427-430"},"PeriodicalIF":0.0,"publicationDate":"2025-06-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/iju5.70059","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144524928","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Takahiro Tsumori, Seiji Hoshi, Kei Yaginuma, Satoru Meguro, Kanako Matsuoka, Junya Hata, Yuichi Sato, Hidenori Akaihata, Soichiro Ogawa, Yoshiyuki Kojima
Introduction
A case of muscle-invasive bladder cancer and renal granulomatosis that developed after intravesical Bacillus Calmette-Guérin therapy, in which a combination of neoadjuvant gemcitabine-cisplatin and anti-tuberculosis therapy was safely administered, and radical cystectomy was ultimately performed, is reported.
Case Presentation
A 64-year-old man with non-muscle-invasive bladder cancer underwent transurethral resection and intravesical Bacillus Calmette-Guérin therapy every time bladder cancer recurred. However, the patient developed left renal granulomatosis during treatment. Anti-tuberculosis therapy was prioritized since there was no bladder cancer progression. However, local bladder cancer progression was observed during the anti-tuberculosis therapy. To successfully cure the renal granulomatosis and suppress tumor progression, neoadjuvant gemcitabine-cisplatin was combined with anti-tuberculosis therapy for 2 months, followed by radical cystectomy. There were no gemcitabine-cisplatin complications and no renal granulomatosis recurrence during combination therapy.
Conclusion
Combination of gemcitabine-cisplatin and anti-tuberculosis therapy was possible for a patient with bladder cancer when Bacillus Calmette-Guérin infection was under control.
{"title":"Combination of Neoadjuvant Gemcitabine-Cisplatin and Anti-Tuberculosis Therapy for a Patient With Muscle-Invasive Bladder Cancer and Renal Granulomatosis That Progressed After Intravesical Bacillus Calmette-Guérin Therapy","authors":"Takahiro Tsumori, Seiji Hoshi, Kei Yaginuma, Satoru Meguro, Kanako Matsuoka, Junya Hata, Yuichi Sato, Hidenori Akaihata, Soichiro Ogawa, Yoshiyuki Kojima","doi":"10.1002/iju5.70057","DOIUrl":"https://doi.org/10.1002/iju5.70057","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>A case of muscle-invasive bladder cancer and renal granulomatosis that developed after intravesical Bacillus Calmette-Guérin therapy, in which a combination of neoadjuvant gemcitabine-cisplatin and anti-tuberculosis therapy was safely administered, and radical cystectomy was ultimately performed, is reported.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case Presentation</h3>\u0000 \u0000 <p>A 64-year-old man with non-muscle-invasive bladder cancer underwent transurethral resection and intravesical Bacillus Calmette-Guérin therapy every time bladder cancer recurred. However, the patient developed left renal granulomatosis during treatment. Anti-tuberculosis therapy was prioritized since there was no bladder cancer progression. However, local bladder cancer progression was observed during the anti-tuberculosis therapy. To successfully cure the renal granulomatosis and suppress tumor progression, neoadjuvant gemcitabine-cisplatin was combined with anti-tuberculosis therapy for 2 months, followed by radical cystectomy. There were no gemcitabine-cisplatin complications and no renal granulomatosis recurrence during combination therapy.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>Combination of gemcitabine-cisplatin and anti-tuberculosis therapy was possible for a patient with bladder cancer when Bacillus Calmette-Guérin infection was under control.</p>\u0000 </section>\u0000 </div>","PeriodicalId":52909,"journal":{"name":"IJU Case Reports","volume":"8 4","pages":"419-422"},"PeriodicalIF":0.0,"publicationDate":"2025-05-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/iju5.70057","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144525176","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Lobular capillary hemangioma is a benign tumor that commonly occurs in the skin and mucous membranes. A lobular capillary hemangioma that occurs within blood vessels is called intravenous lobular capillary hemangioma. In this report, we report the fifth case of intravenous lobular capillary hemangioma originating from a renal vessel.
Case Presentation
A 68-year-old man was diagnosed with right renal cell carcinoma with right renal vein tumor thrombus on computed tomography examination for asymptomatic aortic valve stenosis. The patient underwent a robot-assisted laparoscopic radical right nephrectomy and was diagnosed with intravenous lobular capillary hemangioma.
Conclusion
It is thought that surgical treatment is necessary to differentiate intravenous lobular capillary hemangioma from renal cell carcinoma in preoperative examinations.
{"title":"Intravenous Lobular Capillary Hemangioma in the Renal Vein Mimicking Renal Cancer","authors":"Keisuke Ueki, Hideaki Ito, Takafumi Kabuto, Yasuharu Kaizaki, Ishida Takuya, Tadahiro Kobayashi","doi":"10.1002/iju5.70054","DOIUrl":"https://doi.org/10.1002/iju5.70054","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>Lobular capillary hemangioma is a benign tumor that commonly occurs in the skin and mucous membranes. A lobular capillary hemangioma that occurs within blood vessels is called intravenous lobular capillary hemangioma. In this report, we report the fifth case of intravenous lobular capillary hemangioma originating from a renal vessel.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case Presentation</h3>\u0000 \u0000 <p>A 68-year-old man was diagnosed with right renal cell carcinoma with right renal vein tumor thrombus on computed tomography examination for asymptomatic aortic valve stenosis. The patient underwent a robot-assisted laparoscopic radical right nephrectomy and was diagnosed with intravenous lobular capillary hemangioma.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>It is thought that surgical treatment is necessary to differentiate intravenous lobular capillary hemangioma from renal cell carcinoma in preoperative examinations.</p>\u0000 </section>\u0000 </div>","PeriodicalId":52909,"journal":{"name":"IJU Case Reports","volume":"8 4","pages":"406-410"},"PeriodicalIF":0.0,"publicationDate":"2025-05-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/iju5.70054","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144525174","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
No previous instances of percutaneous transretropubic prostate biopsy have been documented.
Case Presentation
A 74-year-old male patient with a permanent stoma, who had undergone colectomy for descending colon cancer two decades earlier, reported experiencing dysuria. A screening examination revealed an elevated prostate-specific antigen level of 120.24 ng/mL. Despite an intact rectum, the patient's anus was severely constricted or blocked, preventing both digital rectal examination and the insertion of a transrectal ultrasound probe. A transabdominal ultrasound-guided transretropubic prostate biopsy was conducted while monitoring the needle tip position using computed tomography. The subsequent pathological analysis confirmed prostatic adenocarcinoma.
Conclusion
This case represents the first reported instance of a percutaneous transretropubic prostate biopsy.
{"title":"Percutaneous Transretropubic Needle Prostatic Biopsy Under US and CT Guidance","authors":"Maki Hirao, Hideki Ishimaru, Satomi Yoshimi, Takamasa Nishimura, Taiga Oka, Chika Somagawa, Tomoki Nakano, Ryoichi Imamura, Ryo Toya","doi":"10.1002/iju5.70056","DOIUrl":"https://doi.org/10.1002/iju5.70056","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>No previous instances of percutaneous transretropubic prostate biopsy have been documented.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case Presentation</h3>\u0000 \u0000 <p>A 74-year-old male patient with a permanent stoma, who had undergone colectomy for descending colon cancer two decades earlier, reported experiencing dysuria. A screening examination revealed an elevated prostate-specific antigen level of 120.24 ng/mL. Despite an intact rectum, the patient's anus was severely constricted or blocked, preventing both digital rectal examination and the insertion of a transrectal ultrasound probe. A transabdominal ultrasound-guided transretropubic prostate biopsy was conducted while monitoring the needle tip position using computed tomography. The subsequent pathological analysis confirmed prostatic adenocarcinoma.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>This case represents the first reported instance of a percutaneous transretropubic prostate biopsy.</p>\u0000 </section>\u0000 </div>","PeriodicalId":52909,"journal":{"name":"IJU Case Reports","volume":"8 4","pages":"415-418"},"PeriodicalIF":0.0,"publicationDate":"2025-05-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/iju5.70056","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144525175","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Congenital lipoid adrenal hyperplasia (CLAH) is a type of congenital adrenal hyperplasia characterized by reduced steroid production. Patients with this endocrine disorder as well as chromosome 46,XY have testes and female external genitalia. Because these individuals are usually raised as female, the testes are removed.
Case Presentation
A 22-day-old female infant with fever and poor feeding was diagnosed with hypoadrenocorticism. Chromosome test results identified a 46,XY karyotype. Therefore, CLAH was diagnosed. At 1 year and 6 months of age, the patient underwent laparoscopic gonadectomy. Pathological testing of the testes revealed fat deposits in Leydig cells and decreased germ cells.
Conclusion
CLAH with chromosome 46,XY may be complicated by spermatogenesis deficiency.
{"title":"Histopathological Features of the Testes of a Patient With Congenital Lipoid Adrenal Hyperplasia","authors":"Takuya Sakata, Kentaro Mizuno, Daisuke Matsumoto, Hidenori Nishio, Atsushi Suzuki, Kohei Aoyama, Atsushi Ishida, Takahiro Yasui, Haruo Mizuno, Yutaro Hayashi","doi":"10.1002/iju5.70048","DOIUrl":"https://doi.org/10.1002/iju5.70048","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>Congenital lipoid adrenal hyperplasia (CLAH) is a type of congenital adrenal hyperplasia characterized by reduced steroid production. Patients with this endocrine disorder as well as chromosome 46,XY have testes and female external genitalia. Because these individuals are usually raised as female, the testes are removed.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case Presentation</h3>\u0000 \u0000 <p>A 22-day-old female infant with fever and poor feeding was diagnosed with hypoadrenocorticism. Chromosome test results identified a 46,XY karyotype. Therefore, CLAH was diagnosed. At 1 year and 6 months of age, the patient underwent laparoscopic gonadectomy. Pathological testing of the testes revealed fat deposits in Leydig cells and decreased germ cells.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>CLAH with chromosome 46,XY may be complicated by spermatogenesis deficiency.</p>\u0000 </section>\u0000 </div>","PeriodicalId":52909,"journal":{"name":"IJU Case Reports","volume":"8 4","pages":"386-389"},"PeriodicalIF":0.0,"publicationDate":"2025-05-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/iju5.70048","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144525086","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
The prognosis of unresectable metastatic adrenocortical carcinoma is very poor. We report a case of Lynch syndrome accompanying metastatic adrenocortical carcinoma treated with pembrolizumab.
Case Presentation
A 73-year-old woman was diagnosed with left adrenocortical carcinoma and multiple lung, liver, and lymph node metastases. First-line mitotane therapy failed due to toxicity and progressive disease. Immunohistochemical analysis of mismatch repair proteins revealed an MSH6 deficiency. Pembrolizumab monotherapy was started for microsatellite instability-high/mismatch repair–deficient malignant disease. After the first administration, we experienced temporal clinical findings considered to reflect the collapse of tumors. She gained remarkable reductions in all lesions after four cycles. Genetic analysis disclosed the germline pathogenic variant of MSH6, so this case was diagnosed as Lynch syndrome.
Conclusion
We report a patient with metastatic adrenocortical carcinoma in Lynch syndrome who demonstrated an excellent response to pembrolizumab. Genetic analyses can play a beneficial role in cases of adrenocortical carcinoma.
{"title":"An Excellent Clinical and Radiological Response Pattern to Pembrolizumab in a Patient With Metastatic Adrenocortical Carcinoma and Lynch Syndrome","authors":"Yuki Shimozawa, Yosuke Yasuda, Emiko Sugawara, Ryosuke Oki, Kosuke Takemura, Tetsuya Urasaki, Ryo Fujiwara, Noboru Numao, Junji Yonese, Takeshi Yuasa","doi":"10.1002/iju5.70040","DOIUrl":"https://doi.org/10.1002/iju5.70040","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>The prognosis of unresectable metastatic adrenocortical carcinoma is very poor. We report a case of Lynch syndrome accompanying metastatic adrenocortical carcinoma treated with pembrolizumab.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case Presentation</h3>\u0000 \u0000 <p>A 73-year-old woman was diagnosed with left adrenocortical carcinoma and multiple lung, liver, and lymph node metastases. First-line mitotane therapy failed due to toxicity and progressive disease. Immunohistochemical analysis of mismatch repair proteins revealed an MSH6 deficiency. Pembrolizumab monotherapy was started for microsatellite instability-high/mismatch repair–deficient malignant disease. After the first administration, we experienced temporal clinical findings considered to reflect the collapse of tumors. She gained remarkable reductions in all lesions after four cycles. Genetic analysis disclosed the germline pathogenic variant of <i>MSH6</i>, so this case was diagnosed as Lynch syndrome.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>We report a patient with metastatic adrenocortical carcinoma in Lynch syndrome who demonstrated an excellent response to pembrolizumab. Genetic analyses can play a beneficial role in cases of adrenocortical carcinoma.</p>\u0000 </section>\u0000 </div>","PeriodicalId":52909,"journal":{"name":"IJU Case Reports","volume":"8 4","pages":"361-364"},"PeriodicalIF":0.0,"publicationDate":"2025-05-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/iju5.70040","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144524784","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Bladder rupture is a rare complication of kidney transplantation, with an increased risk in patients with low-compliance bladders.
Case Presentation
A man in his 40s with end-stage renal disease secondary to diabetic nephropathy underwent ABO-incompatible living-donor kidney transplantation. He had been anuric for 3.5 years. Bladder rupture was identified during saline injection into the bladder before ureterovesical anastomosis was performed. It was repaired using full-thickness sutures, and the surgical approach was changed from ureterovesical anastomosis to ureteroureterostomy. The catheter was removed on POD 21, and voiding improved over time without complications.
Conclusion
In patients with bladder atrophy, preventing sudden increases in intravesical pressure and ensuring careful catheter handling are crucial for avoiding bladder rupture.
{"title":"A Case of Intraoperative Bladder Rupture During Kidney Transplantation","authors":"Kosuke Ogawa, Yusuke Tabuchi, Takahiro Yamaguchi, Ryo Iguchi, Hitomi Miyata, Noriyuki Ito, Kazutoshi Okubo","doi":"10.1002/iju5.70053","DOIUrl":"https://doi.org/10.1002/iju5.70053","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>Bladder rupture is a rare complication of kidney transplantation, with an increased risk in patients with low-compliance bladders.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case Presentation</h3>\u0000 \u0000 <p>A man in his 40s with end-stage renal disease secondary to diabetic nephropathy underwent ABO-incompatible living-donor kidney transplantation. He had been anuric for 3.5 years. Bladder rupture was identified during saline injection into the bladder before ureterovesical anastomosis was performed. It was repaired using full-thickness sutures, and the surgical approach was changed from ureterovesical anastomosis to ureteroureterostomy. The catheter was removed on POD 21, and voiding improved over time without complications.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>In patients with bladder atrophy, preventing sudden increases in intravesical pressure and ensuring careful catheter handling are crucial for avoiding bladder rupture.</p>\u0000 </section>\u0000 </div>","PeriodicalId":52909,"journal":{"name":"IJU Case Reports","volume":"8 4","pages":"402-405"},"PeriodicalIF":0.0,"publicationDate":"2025-05-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/iju5.70053","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144524781","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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