Labial synechiae: a diagnostic conundrum.

The purpose of this study was to shed some light on labial synechiae, which is a common yet unfamiliar condition seen initially by the family physician and ultimately by the paediatric urologist. It is often diagnosed incorrectly, leading to undue anxiety and stress for the unsuspecting parents, and results in multiple unnecessary lab investigations, hence, putting a burden on the healthcare system in general. After IRB approval, a retrospective chart review was performed over a period of 15 years (2007-2021), at The Indus Hospital, Karachi, Pakistan. The records of all female children who underwent examination under anaesthesia (EUA) for labial synechiae (n=29) were included in the study. Our results indicate that the primary healthcare physicians were unable to recognise labial adhesions at the time of initial presentation. We conclude that labial synechiae is a benign condition affecting female infants, but is not very well understood by healthcare workers in our part of the world.

JRODOS customization for Pakistan and assessment of the consequences of a hypothetical nuclear accident.

Since the Chernobyl disaster in 1986, decision support systems and modelling tools have been utilized in response to nuclear and radiological emergencies. The java-based real-time online decision support system (JRODOS) is a decision support tool that can be utilised in response to an emergency in managing off-site radiological consequences. This article documents the customization and use of JRODOS for Pakistan. JRODOS was tailored to the local Pakistan conditions, and a case study of a theoretical nuclear power plant accident was used to assess JRODOS's feasibility as a decision support tool. A worst-case probabilistic accident scenario was used to identify zones and areas where urgent protective actions, early protective actions and food restriction and other response actions could be required. The areas and distances identified for the implementation of protective and response actions for such a hypothetical accident were found to be in agreement with the emergency planning zones and distances suggested by the International Atomic Energy Agency (IAEA). Additionally, the implications of meteorological and source term input parameters on predicting the radiation doses to members of the public were investigated. It was identified that the output of such tools strongly depends on the availability and accuracy of the input parameters, such as radioactive release and meteorological data. Limitations and uncertainties associated with these tools need to be considered in deciding on protective and other response actions in response to a nuclear accident. As established by the IAEA, protective and other response actions need to be applied on a graded approach, taking into account the protection strategy and uncertainties and limitations in the available information and criteria, based on the conditions at the facility and off-site.

Paediatric penile perception score (PPPS) in hypospadias repairde34.

The present cross-sectional study sought to evaluate the Paediatric Penile Perception Score (PPPS) as a tool to assess parental perception of hypospadias repair in children. In all, 60 patients were enrolled in the study with median age of 3.9 years; of these 24 (40%) had proximal hypospadias and 36 (60%) had distal hypospadias. After hypospadias repair, parents/guardians were interviewed about meatus, glans, skin, and general appearance using a standardised questionnaire to report penile appearance and penile perception. Overall, the median PPPS score was 17 (IQR: 16-18) with no significant difference in the penile perception score between the type of hypospadias, relationship of the person interviewed with the child (mother, father or other), nor the children's age. The study revealed that penile perception score (PPPS) is a valuable tool that can be used to assess parental penile perception regarding the child's penile appearance and noted overall high satisfaction after hypospadias repair.

The C-terminus of Gain-of-Function Mutant p53 R273H Is Required for Association with PARP1 and Poly-ADP-Ribose.

The TP53 gene is mutated in 80% of triple-negative breast cancers. Cells that harbor the hot-spot p53 gene mutation R273H produce an oncogenic mutant p53 (mtp53) that enhances cell proliferative and metastatic properties. The enhanced activities of mtp53 are collectively referred to as gain-of-function (GOF), and may include transcription-independent chromatin-based activities shared with wild-type p53 (wtp53) such as association with replicating DNA and DNA replication associated proteins like PARP1. However, how mtp53 upregulates cell proliferation is not well understood. wtp53 interacts with PARP1 using a portion of its C-terminus. The wtp53 oligomerization and far C-terminal domain (CTD) located within the C-terminus constitute putative GOF-associated domains, because mtp53 R273H expressing breast cancer cells lacking both domains manifest slow proliferation phenotypes. We addressed if the C-terminal region of mtp53 R273H is important for chromatin interaction and breast cancer cell proliferation using CRISPR-Cas9 mutated MDA-MB-468 cells endogenously expressing mtp53 R273H C-terminal deleted isoforms (R273HΔ381-388 and R273HΔ347-393). The mtp53 R273HΔ347-393 lacks the CTD and a portion of the oligomerization domain. We observed that cells harboring mtp53 R273HΔ347-393 (compared with mtp53 R273H full-length) manifest a significant reduction in chromatin, PARP1, poly-ADP-ribose (PAR), and replicating DNA binding. These cells also exhibited impaired response to hydroxyurea replicative stress, decreased sensitivity to the PARP-trapping drug combination temozolomide-talazoparib, and increased phosphorylated 53BP1 foci, suggesting reduced Okazaki fragment processing. IMPLICATIONS: The C-terminal region of mtp53 confers GOF activity that mediates mtp53-PARP1 and PAR interactions assisting DNA replication, thus implicating new biomarkers for PARP inhibitor therapy.

Evaluation of adverse effects of particulate matter on human life.

Particulate matter (PM2.5) has a severe impact on human health. The concentration of PM2.5, related to air-quality changes, may be associated with perceptible effects on people's health. In this study, computer intelligence was used to assess the negative effects of PM2.5. The input data, used for the evaluation, were grid definitions (shape-file), PM2.5, air-quality data, incidence/prevalence rates, a population dataset, and the (Krewski) health-impact function. This paper presents a local (Pakistan) health-impact assessment of PM2.5 in order to estimate the long-term effects on mortality. A rollback-to-a-standard scenario was based on the PM2.5 concentration of 15 µg m-3. Health benefits for a population of about 73 million people were calculated. The results showed that the estimated avoidable mortality, linked to ischemic heart disease and lung cancer, was 2,773 for every 100,000 people, which accounts for 2,024,290 preventable deaths of the total population. The total cost, related to the above mortality, was estimated to be US $ 1,000 million. Therefore, a policy for a PM2.5-standard up to 15 µg m-3 is suggested.

Urothelial carcinoma in children, case report with review of literature.

Urothelial carcinoma is the third most prevalent malignancy in adults, accounting for 2.1 % of all cancerrelated deaths. The highest incidence in the 6th decade of life but it is rare in the paediatric population and its incidence is less than 1% constituting a very rare disease. We report the case of a 13 years old boy, who presented to the outpatient department at The Indus Hospital, Karachi, in May 2017, with the complaint of painless gross haematuria.

Post operative pain control in percutaneous nephrolithotomy.

OBJECTIVE: To evaluate post-surgery pain relief in percutaneous nephrolithotomy cases. METHODS: The case-control study was conducted at Indus Hospital, Karachi, from July 2014 to July 2015, and comprised patients undergoing percutaneous nephrolithotomy. The patients were randomized by snose protocol into two equal groups of cases and controls. The cases were given peri-tubal local anaesthetic infiltration of 0.25% bupivacaine, while the controls were given 10ml 0.9% normal saline infiltration. Post-operative pain was assessed using visual analogue scale. RESULTS: Of the 68 patients, there were 34(50) in each of the two groups. Pain score was significantly less in the intervention group post-operatively at 6th and 12th hours (p<0.05). Also, total consumption of analgesics and demand of analgesic medication were also significantly low among the cases (p<0.05). CONCLUSIONS: Bupivacaine infiltration into the peri-tubal track significantly reduced post-operative pain.

Development, validity and reliability of an URDU version of the International Prostate Symptom Score.

OBJECTIVE: To develop and validate the Urdu version of the International Prostate Symptoms Score. METHODS: The prospective study was conducted at Indus Hospital, Karachi, from January to April 2014, and comprised interviews of subjects at baseline and at 2 weeks for assessing the test-retest reliability. Patients presenting with lower urinary track symptoms and who were able to read Urdu were recruited as subjects. The reliability of the Urdu version of the International Prostate Symptoms Score was assessed by determining the internal consistency, and by assessing the test-retest reliability. RESULTS: There were 58 subjects with a mean age of 61.53+/-9.99 years. There was no statistically significant difference in self-administered and assister filling total score (p=0.145) and quality of life (p=0.201). For reliability analysis, Cronbach's alpha coefficient was 0.756 total score and 0.622 for quality of life. CONCLUSIONS: The Urdu version of the International Prostate Symptoms Score stood validated for assessing lower urinary track symptoms.

Leiomyoma of the urinary bladder.

Leiomyoma of urinary bladder is a rare tumour with an incidence of < 0.5% of all the bladder tumours. We report a case of leiomyoma of bladder after taking written and informed consent from a 38 years old female, after obtaining the permission of the institutional review board (IRB). She presented in November 2015, with symptoms of obstructive lower urinary tract for 1 year followed by acute urinary retention. Physical examination was unremarkable. Ultrasound showed well circumscribed polypoidal growth in the bladder with mild hydronephrosis on the left side. CT scan abdomen and pelvis with contrast was done, which revealed well circumscribed, rounded soft tissue lesion of 4.4 x 4.5 cm at the posterior inferior surface of the bladder near the bladder neck. Initially TURBT was done but after 6 months patient again went into urinary retention so local excision of the tumour was done. Histopathology confirmed the diagnosis of leiomyoma of the bladder. The diagnosis and management of leiomyoma of the bladder is presented along with review the literature.

CT-guided Transosseous Soft Tissue Biopsy: Techniques, Outcomes and Complications in 50 Cases.

PURPOSE: To describe the techniques, outcomes and complications of CT-guided transosseous biopsy of soft tissue lesions via multiple different routes. MATERIALS AND METHODS: Clinical and radiologic data from all consecutive soft tissue biopsies performed via a transosseous approach between April 2009 and July 2015 were retrospectively compiled. Fifty biopsies performed in 50 patients (n = 17 males, n = 33 females) were included. Biopsies were performed using a 13-gauge biopsy needle which was advanced through the selected bone to the lesion margin followed by coaxial placement of either an 18- or 20-gauge biopsy gun. Sampling accuracy was determined from the final pathology report as diagnostic or non-diagnostic. Complications included rates of pneumothorax, pneumomediastinum, hemoptysis, immediate admission, chest tube insertion, surgical consultation, 30-day readmission, fracture, osteomyelitis or tract seeding. RESULTS: Number of diagnostic samples per bone included: sternum (n = 17, 34%), rib (n = 7, 14%), scapula (n = 6, 12%), transverse process (n = 4, 8%), vertebral body (n = 4, 8%), spinous process (n = 1, 2%), ilium (n = 7, 14%), sacrum (n = 4, 8%). Complications included 10 pneumothoraces in 39 cases that crossed the pleura, 8 pneumomediastinum in 17 transsternal cases, one immediate surgical consultation and one 30-day readmission. CONCLUSION: Transosseous biopsy approach is a technically feasible means of obtaining core needle samples of soft tissue lesions. LEVEL OF EVIDENCE: Case series, IV.

Radiologic Assessment of Native Renal Vasculature: A Multimodality Review.

A wide range of clinically important anatomic variants and pathologic conditions may affect the renal vasculature, and radiologists have a pivotal role in the diagnosis and management of these processes. Because many of these entities may not be suspected clinically, renal artery and vein assessment is an essential application of all imaging modalities. An understanding of the normal vascular anatomy is essential for recognizing clinically important anatomic variants. An understanding of the protocols used to optimize imaging modalities also is necessary. Renal artery stenosis is the most common cause of secondary hypertension and is diagnosed by using both direct ultrasonographic (US) findings at the site of stenosis and indirect US findings distal to the stenosis. Fibromuscular dysplasia, while not as common as atherosclerosis, remains an important cause of renal artery hypertension, especially among young female individuals. Fibromuscular dysplasia also predisposes individuals to renal artery aneurysms and dissection. Although most renal artery dissections are extensions of aortic dissections, on rare occasion they occur in isolation. Renal artery aneurysms often are not suspected clinically before imaging, but they can lead to catastrophic outcomes if they are overlooked. Unlike true aneurysms, pseudoaneurysms are typically iatrogenic or posttraumatic. However, multiple small pseudoaneurysms may be seen with underlying vasculitis. Arteriovenous fistulas also are commonly iatrogenic, whereas arteriovenous malformations are developmental (ie, congenital). Both of these conditions involve a prominent feeding artery and draining vein; however, arteriovenous malformations contain a nidus of tangled vessels. Nutcracker syndrome should be suspected when there is distention of the left renal vein with abrupt narrowing as it passes posterior to the superior mesenteric artery. Filling defects in a renal vein can be due to a bland or tumor thrombus. A tumor thrombus is most commonly an extension of renal cell carcinoma. When an enhancing mass is located predominantly within a renal vein, leiomyosarcoma of the renal vein should be suspected. ©RSNA, 2017.

Renal aspergilloma presenting with pelvi-ureteric junction Obstruction (PUJO).

Primary renal aspergillosis, though a rare entity, is still seen in immune compromised individuals. Renal aspergillosis may lead to formation of focal abscesses, fungal bezoars and may cause ureteric obstruction. Treatment involves stabilization of patient and removal of fungal bezoars along with administration of anti-fungal agents. This report describes the case of localized primary renal aspergillosis with fungal bezoars formation in a 55 years old female, diabetic, hypertensive, who presented with upper urinary tract obstruction and was successfully managed by endoscopic removal of fungal bezoars and intravenous amphotericin followed by oral itraconazole.

Adrenal myelolipoma in a young male - a rare case scenerio.

Adrenal myelolipomas are rare non functional benign tumours which are incidentally diagnosed in asymptomatic patients. Despite the fact that several theories have been discussed, the origin of myelolipoma remains unclear. Our patient, 19 years old male presented with history of abdominal pain for past 7 years. Computed tomography scan of abdomen with oral and intravenous contrast revealed well defined round heterogenous enhancing mass seen in right supra renal area involving the adrenal gland. Patient underwent surgery by open access through thoracoabdominal approach. Histopathology of resected mass showed myelolipoma along with trilineage haematopoesis with mature adipose tissues.

How to improve outcome in surgery for Proximal Hypospadias?

OBJECTIVE: To evaluate the role of subdartos fascial tissue as watertight layer in improving outcome for 2-stage proximal hypospadias surgery. METHODS: The experimental study was conducted at the Department of Urology, Indus Hospital, Karachi, and comprised an audit of patients with proximal hypospadias who underwent surgery from July 1, 2007, to December 31, 2011. The initial two-stage repair of proximal hypospadias led to a high rate of urethrocutanous fistula formation (Group A), and, thus, a modification was introduced and subdartos facial double layer was applied over the urethral suture line (Group B). The results were compared regarding age, type of hypospadias, graft failure and urethrocutanous fistula in these patients. RESULTS: There were 27 patients in Group A and 16(59.3%) of them ended up having urethrocutanous fistula. Group B had 25 patients and only 2(8%) had fistula formation. CONCLUSIONS: The application of dartos facial flap waterproofing layer reduced fistula rate.

Imaging evaluation of the inferior vena cava.

The inferior vena cava (IVC) is an essential but often overlooked structure at abdominal imaging. It is associated with a wide variety of congenital and pathologic processes and can be a source of vital information for referring clinicians. Initial evaluation of the IVC is most likely to occur at computed tomography performed for another indication. Many routine abdominal imaging protocols may result in suboptimal evaluation of the IVC; however, techniques to assist in specific evaluation of the IVC can be used. In this article, the authors review the spectrum of IVC variants and pathologic processes and the relevant findings from magnetic resonance imaging, angiography, sonography, and positron emission tomography. Embryologic development of the IVC and examples of congenital IVC variants, such as absence, duplication, left-sided location, azygous or hemiazygous continuation, and web formation, are described. The authors detail IVC involvement in Wilms tumor, leiomyosarcoma, adrenal cortical carcinoma, testicular carcinoma, hepatocellular carcinoma, renal cell carcinoma, and other neoplasms, as well as postsurgical, traumatic, and infectious entities (including filter malposition, mesocaval shunt, and septic thrombophlebitis). The implications of these entities for patient treatment and instances in which specific details should be included in the dictated radiology report are highlighted. Furthermore, the common pitfalls of IVC imaging are discussed. The information provided in this review will allow radiologists to detect and accurately characterize IVC abnormalities to guide clinical decision making and improve patient care.

Adrenocortical oncocytoma.

Adrenocortical oncocytoma is an exceedingly rare pathological variant of an adrenal neoplasm. The pathogenesis of oncocytic neoplasms is poorly understood. Females have been reported to be affected 2.5 times more frequently than males, and left-sided lesions are more common than those on right side. This case describes a 20 years old female with right lumbar pain. She found to have a right adrenal gland mass measuring about 6 x 5 cm. A computed tomogram showed hypodense mass lesion 6 x 4.2 cm involving right adrenal gland. Differential diagnosis of non-functional adrenal adenoma was made. A laparoscopic right adrenalectomy was performed using the 3-ports lateral transperitoneal approach. Histopathology showed adrenocortical oncocytoma of adrenal gland.

Segmental testicular infarction: sonographic findings and pathologic correlation.

Segmental testicular infarction can mimic testicular carcinoma on sonography and can lead to unnecessary orchiectomy. This case series describes and correlates sonographic and histologic findings of 7 pathologically proven segmental testicular infarction cases. Segmental testicular infarction should be suspected on sonography when a geographic lesion with low or mixed echogenicity has absent or near-absent flow in a patient with scrotal pain. A hyperechoic rim and peripheral hyperemia correspond to interstitial hemorrhage and inflammatory changes. As an infarct evolves, it becomes more discrete and hypoechoic as ghost outlines replace seminiferous tubules. Follow-up or contrast-enhanced magnetic resonance imaging or sonography can increase diagnostic confidence in suspected cases and prevent unnecessary orchiectomy.

Imaging of non-neoplastic intratesticular masses.

The use of high-frequency ultrasound is increasing for the treatment of cystic, vascular, and solid non-neoplastic intratesticular masses. Cystic lesions examined include simple testicular cysts, tunica albuginea cysts, epidermoid cysts, tubular ectasia of rete testis, and intratesticular abscesses. Vascular lesions examined include intratesticular varicocele and intratesticular arteriovenous malformations. Solid lesions examined include fibrous pseudotumor of the testis, focal or segmental testicular infarct, fibrosis of the testis, testicular hematoma, congenital testicular adrenal rests, tuberculoma, and sarcoidosis. Gray-scale and color-flow Doppler sonography facilitate the visualization of the benign characteristics of the lesions. Magnetic resonance imaging can also help as a problem-solving modality in some cases.

Cyclic AMP post-transcriptionally regulates the biosynthesis of a major bacterial autoinducer to modulate the cell density required to activate quorum sensing.

In Vibrio cholerae, expression of the quorum sensing regulator HapR is induced by the accumulation of a major autoinducer synthesized by the activity of CqsA. Here we show that the cAMP-cAMP receptor protein complex regulates cqsA expression at the post-transcriptional level. This conclusion is supported by the analysis of cqsA-lacZ fusions, the ectopic expression of cqsA in Deltacrp mutants and by Northern blot analysis showing that cqsA mRNA is unstable in Deltacrp and Deltacya (adenylate cyclase) mutants. Addition of cAMP to the culture of a Deltacya mutant restored cqsA mRNA stability and cholera autoinducer 1 production. Lowering intracellular cAMP levels by addition of d-glucose increased the cell density required to activate HapR. These results indicate that cAMP acts as a quorum modulator.

Ureteroscopy under spinal versus general anaesthesia: morbidity and stone clearance.

OBJECTIVE: To compare morbidity and stone clearance by ureteroscopy carried out under spinal anaesthesia with that carried out under general anaesthesia. STUDY DESIGN: Quasi-experimental study. PLACE AND DURATION OF STUDY: The study was conducted at the Kidney Centre, Postgraduate Training Institute, Karachi, from September 2005 to December 2006. PATIENTS AND METHODS: A total number of 60 patients were enrolled for the study. All patients underwent ureteroscopic procedure for a calculus disease, using a semi-rigid ureteroscope of 10 Fr. These patients were divided in 2 groups of 30 patients, receiving general or spinal anaesthesia. Operative time, stone clearance, hospital stay, intra- and postoperative complications and visual analogue score for pain were noted in each case. RESULTS: There were 30 patients each in both groups, with 73% males and 27% females in group A (general anaesthesia) and 93% males and 7% females in group B (spinal anaesthesia). Mean age of the patients were 33.9 and 36.9 years in groups A and B respectively. The average stone size was 0.79 cm in group A and 1.14 cm in group B (p=0.001). Duration of surgery was 41.4+/-1.29 minutes in group A and 30.5+/-2.13 minutes in group B (p=0.033). Mean hospital stay was 21.6 and 18.1 hours in group A and B respectively (p=0.073). Mean visual analogue score for pain in group A was 3.1 and group B was 1.8. CONCLUSION: Ureteroscopy under spinal anaesthesia resulted in decreased length of operative time and shortened hospital stay in this series and did not carry any additional risk of major complications.