Notes

A countrywide future pc registry regarding bortezomib-based treatment in light archipelago () amyloidosis.
In this article, we will review the pathophysiology, prevalence, risk factors, clinical implications, and management of spontaneous and iatrogenic hypoglycemia in patients with CF.In India, the major drivers of both internal and international migration are the prevailing unemployment, competitive labour market and enhanced livelihood prospects in the destination state or country. However, the nationwide lockdown and the sealing of inter-state and international borders to control the COVID-19 pandemic triggered the reverse migration of informal migrant workers. This requires the central and state governments to collectively forge strategies to enable their reverse migration and smooth reintegration in the post-COVID economy. In this paper, we have focused on the inter-state migrants in India and returnee migrants only from Gulf countries as they account for two-thirds of Indian migrants living abroad. This study conducted a comparative analysis of the Indian government's varied approach towards its internal and international migrants during their reverse migration, repatriation and reintegration after the announcement of the lockdown. Firstly, the paper compares the challenges faced by internal and international migrant workers during these stages with the help of in-depth interview data collected from migrants and social workers. Secondly, the varied governmental responses towards their repatriation are discussed. Thirdly, it analyses the obstacles in their economic reintegration to help frame suitable welfare policies for the Indian migrant community.Vesicovaginal fistula with stone is an infrequent condition; therefore, it can be often misdiagnosed. The formation of vaginal stones is mainly due to urinary stasis and infection. We describe the case of a 69-year-old patient who was diagnosed with a vesicovaginal fistula in 2012. The etiology of the disease was related to supravesical amputation of the uterus and radiation therapy. 9 years later a medium-sized stone was found in the fistula. This type of case is rare in practice. The final diagnosis can be made together with gynecologists based on the results of pelvic computed tomography.The barbed suture e.g. V-Loc™, are occasionally used in urological surgery due to the practical advantage of omitting the need for surgical knots and reducing surgical time. In this report, an 83-year-old man diagnosed as having invasive bladder cancer underwent robot-assisted radical cystectomy on the thirteenth postoperative day, he was diagnosed as having strangulated ileus of the small bowel. The small bowel was strangulated by bands formed between the stump of V-Loc™ and the fatty appendices of the sigmoid colon. This is the first report of a small bowel ileus associated with the barbed suture in the field of urology.Angioleiomyoma (vascular leiomyoma) is a common benign smooth muscle tumor, often affecting the soft tissue of the extremities, head, and neck regions. However, testicular involvement is an exceedingly rare event. Herein, we present an unusual case of testicular angioleiomyoma. To the best of our knowledge, this is the third reported case in English literature.Herlyn-Werner-Wunderlich (HWW) syndrome is difficult to diagnose. We report our first experience of fistula repair in HWW syndrome in Hasan Sadikin General Hospital Indonesia. A 12 years old girl presented with urinary retention, and was consulted to urology because the urethral meatus could not be found. MRI showed two separate uteri, cervices, and vaginas. Complete separation of vagina and urethra was done. After 2 years follow up, the patient had no complaint. HWW syndrome should be suspected in cases with pelvic pain or urinary retention during menarche period among teenagers and neonatal cases with any renal malformation.Transverse testicular ectopia(TTE) is a rare anomaly defined by the presence of both testis in the same hemiscrotum or in the same inguinal canal.The main treatment of TTE is surgical intervention. Here we report a case of type 2 transverse testicular ectopia in an eight months old child. The boy had a right side inguinal hernia and a non-palpable testis on the left side. Ultrasonography confirmed the presence of both testis at the right inguinal region. Our case was associated with the persistence of Mullerian duct. After the excision of the Mullerian duct remnant, bilateral orchiopexy was performed.Testicular varicocele is a dilatation of the pampiniform plexus. It is a common clinical problem affecting 15% of adolescents and adults that may be associated with pain and discomfort, and can affect fertility. Varicocele treatment is associated with a significant improvement in sperm parameters, and pregnancy rate. For endovascular treatment Fibered embolization coils are commonly utilized. Complications associated with coil insertion are rare, but can have serious consequences. Here, we presenting the first reported case of a retroperitoneal abscess following testicular vein coil embolization. The patient underwent successful laparoscopic removal the infected coil followed by full recovery.A 67-year-old man underwent RARP in the Trendelenburg position with pneumoperitoneum at 12 mmHg. Gradual elevation of End-tidal CO2(EtCO2) began, and extensive subcutaneous emphysema was recognized when EtCO2 reached 58 mmHg. After interruption of pneumoperitoneum, careful observation of the surgical field led to detect an injury of the abdominal wall of 1 cm in length, suggesting the cause of severe subcutaneous emphysema. The injury was repaired and RARP was terminated without any cardiovascular problems. Attention should be paid that even minor abdominal wall injury could lead to severe subcutaneous emphysema which may cause respiratory or cardiovascular problems during laparoscopic surgeries.Congenital urethral polyps are a rare anomaly of the urethra seen primarily in male children that present most commonly with features of voiding dysfunction or urinary obstruction. We report a case of a young boy with a posterior urethral polyp presenting with urinary retention, hematuria, and constipation. We also report a new treatment approach to this rare and challenging clinical finding.We report a case of leukocytoclastic vasculitis-induced penile necrosis in a 69-year-old male with no previous urologic history. After suffering an ischemic event to the distal shaft of the penis as well as an ischemic event involving the right side of the bladder, he underwent suprapubic tube placement for urinary diversion. Despite attempts at anticoagulation for penile salvage, he lost the distal aspect of the glans penis to auto-necrosis. Ultimately, the underlying disease was determined to be systemic ANCA-associated vasculitis, and the patient was treated with rituximab and prednisone as well as penile wound debridement.The urinary bladder is the most common site of foreign bodies in the genitourinary tract. The incidence of admissions related to this appears to be increasing, which may be partly due to an increase in the practice of urethral sounding for sexual gratification. Herein, a 29 year-old lady who was using a urethral sound for sexual arousal when it slipped and migrated into the bladder. We describe a technique that can be used to retrieve cylindrical foreign bodies from the bladder using an Endoloop through a rigid cystoscope, which has been found to be relatively atraumatic and easy to perform.Primary neuroendocrine differentiation in prostate cancer occurs infrequently and represents a therapeutic challenge at present due to the poor prognosis involved. We present the case of a patient with de novo neuroendocrine prostate cancer who later developed metastases to adrenals bilaterally which were initially managed surgically.Penile duplication is a very rare urological entity. It may be associated with other congenital conditions such as urogenital, GI tract and musculoskeletal anomalies. Properly classifying the condition may dictate the final treatment options. Our current case is the complete true duplication in which we performed side-to-side urethra-urethral anastomosis. We spared the posterior urethra as it may end up with postoperative urinary incontinence.
Dihydrolipoamide dehydrogenase deficiency (DLDD) is a rare metabolic disorder inherited in an autosomal recessive manner. This heterogeneous disease has a variable clinical presentation, onset, and biochemical markers.

We retrospectively reviewed the clinical and molecular diagnosis of eight cases with DLDD from four referral centers in Saudi Arabia.

Remarkably, we found hepatic involvement ranging from acute hepatic failure to chronic hepatitis in five patients. In addition, neurological disorders in the form of seizures, developmental delay, ataxia, hypotonia and psychomotor symptoms were found in five patients, two of them with a combination of hepatic and neurological symptoms. In addition, only one patient had recurrent episodes of hypoglycemia. While most patients had the hepatic form of homozygous variant c.685G>T in the
gene, one patient was found to have a novel variant c.623C>T that had neurological and hepatic symptoms


We describe the largest reported DLDD cohort in the Saudi population. Clinical, biochemical, radiological, and molecular characterization was reviewed and no clear genotype-phenotype correlation was found in this cohort.
We describe the largest reported DLDD cohort in the Saudi population. Clinical, biochemical, radiological, and molecular characterization was reviewed and no clear genotype-phenotype correlation was found in this cohort.We investigated the decline of activities of daily living with symptomatic progression in patients with mucopolysaccharidosis type II (MPS II) and investigated the associated factors. Clinical data were retrospectively collected from the medical records of 28 patients with MPS II who visited our hospital between October 2007 and August 2019. Activities of daily living were assessed over time using a 5-point scale (from stage 1, indicating independent, to stage 5, indicating total assistance + medical care); the relationships of the interval years from stage 2 (mild symptoms) to stage 4 (total assistance) with therapeutic intervention, anti-drug antibodies (ADA), urinary glycosaminoglycans (uGAG), and genotypes were analyzed. Eight are attenuated types, and 20 are severe types. buy CX-5461 Further, 20 underwent enzyme replacement therapy (ERT) alone, 5 underwent hematopoietic stem cell transplantation (HSCT) alone, and 3 underwent both therapy. The mean interval years (standard deviation) from stage 2 to 4 was 3.5 (0.7) and 7.3 (3.3) in patients who started undergoing ERT (n = 6) and HSCT (n = 3) at stage 2, respectively, whereas it was 3.1 (1.5) in patients who received no treatment until they reached stage 4 (n = 8). The study findings revealed the process of changes in the activities of daily living over a long duration in patients with MPS II undergoing different treatments. In severe type, the activity deteriorated regardless of the stage at which ERT was initiated. The activity declined slower in patients who received HSCT at an early stage.
To characterize the prevalence of brain ischemia and cerebral small vessel disease in a cohort of patients with Fabry disease (FD) seen at an academic medical center.

FD is an inherited X-linked lysosomal storage disorder with central nervous system involvement. Limited data are available in the literature on the cerebrovascular neuroimaging findings in FD, and the reported prevalence of stroke symptoms and cerebral small vessel disease has varied widely.

Brain MRI was performed in 21 patients with FD followed at University of California Irvine Medical Center. Stroke symptoms were assessed and quantification of cerebral microvascular disease was performed using small vessel disease (SVD) score. Lacunes and deep white matter hyperintensities were scored on a four-point scale of 0 (absent) and 1-3 to account for increasing severity; microbleeds were scored 0 (absent) or 1 (present). The total SVD score is the sum of the three components and ranges from 0 to 7.

Nearly 43% (9/21) of our FD cohort (aged 32-81years, mean=50) had a SVD score of one or higher, all of whom were aged 50 or more years.
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