Obstructive or non-obstructive megacystis: a prenatal dilemma.

Introduction: Diagnosis of prenatal megacystis has a significant impact on the pregnancy, as it can have severe adverse effects on fetal and neonatal survival and renal and pulmonary function. The study aims to investigate the natural history of fetal megacystis, to try to differentiate in utero congenital lower urinary tract obstruction (LUTO) from non-obstructive megacystis, and, possibly, to predict postnatal outcome. Materials and methods: A retrospective single-center observational study was conducted from July 2015 to November 2023. The inclusion criteria were a longitudinal bladder diameter (LBD) >7 mm in the first trimester or an overdistended/thickened-walled bladder failing to empty in the second and third trimesters. Close ultrasound follow-up, multidisciplinary prenatal counseling, and invasive and non-invasive genetic tests were offered. Informed consent for fetal autopsy was obtained in cases of termination of pregnancy or intrauterine fetal demise (IUFD). Following birth, neonates were followed up at the same center. Patients were stratified based on diagnosis: LUTO (G1), urogenital anomalies other than LUTO ("non-LUTO") (G2), and normal urinary tract (G3). Results: This study included 27 fetuses, of whom 26 were males. Megacystis was diagnosed during the second and third trimesters in 92% of the fetuses. Of the 27 fetuses, 3 (11.1%) underwent an abortion, and 1 had IUFD. Twenty-three newborns were live births (85%) at a mean gestational age (GA) of 34 ± 2 weeks. Two patients (neonates) died postnatally due to severe associated malformations. Several prenatal parameters were evaluated to differentiate patients with LUTO from those with non-LUTO, including the severity of upper tract dilatation, keyhole sign, oligohydramnios, LBD, and GA at diagnosis. However, none proved predictive of the postnatal diagnosis. Similarly, none of the prenatal parameters evaluated were predictive of postnatal renal function. Discussion: The diagnosis of megacystis in the second and third trimesters was associated with live births in up to 85% of cases, with LUTO identified as the main cause of fetal megacystis. This potentially more favorable outcome, compared to the majority reported in literature, should be taken into account in prenatal counseling. Megacystis is an often misinterpreted antennal sign that may hide a wide range of diagnoses with different prognoses, beyond an increased risk of adverse renal and respiratory outcomes.

The digital era of pediatric urological clinical care: telemedicine for management of lower urinary tract symptoms and nocturnal enuresis - a comprehensive tertiary center insight.

BACKGROUND: Lower urinary tract symptoms (LUTS) and nocturnal enuresis (NE) are complex conditions requiring a long-term follow-up. Telemedicine is an emerging technological tool in the surgical field, and its availability exponentially grew during the COVID-19 pandemic, expanding its application fields, optimizing technical aspects, reducing costs, and ensuring high-quality standards. This work describes our experience with telemedicine in a Division of Pediatric Urology for the follow-up of enuresis and LUTS. METHODS: A retrospective analysis of our telemedicine preliminary experience was conducted at Regina Margherita Children's Hospital in Turin, Italy. This study included all the patients aged <18 years who were monitored for enuresis and LUTS through telemedicine between September 1, 2021 and July 31, 2023. Clinical data and outcomes were analyzed, and patients and families were asked to voluntary fill an evaluation questionnaire on their satisfaction. Additionally, we focused on the post COVID-19 period, between September 2022 and July 2023, analyzing the data of two different patients' populations: the first one (G1) of patients choosing telemedicine outpatients visits (TOVs) and the second one (G2) of those choosing a face-to-face visit. RESULTS: One hundred five patients were enrolled. One hundred sixty-two TOVs were performed. The median age at first visit was 9.7±0.66 years (range 7-16 years). Diagnosis were: 77/105 (67%) NE and 28/105 (33%) LUTS. The average referred distance between the patients' residence and the hospital was 46.35±129.37 km (range 2-1300 kilometers) and the time taken to overcome it was 44.21±77.29 minutes (range 10-780 minutes). In 64/105 cases (61%) the follow-up was interrupted for total healing or symptoms resumption. Only two cases (2%) required the conversion to an in-person ambulatory consult, due to a social-linguistic barrier. 146/162 families (90%) filled the survey questionnaire at the end of each TOV, reporting in 94% of cases a high satisfaction level. In the comparative statistical analysis of the two patient groups, G1 (52 telemedicine office visits, [TOVs]) vs. G2 (25 face-to-face visits), the average referred distance was 17.78±7.98 km (range: 5-35 km) for G1, contrasting with 7.04±3.35 km (range: 2.5-14 km) for G2 (P=0.00001). Additionally, the waiting time before the visit was 3.96±2.90 minutes (range: 0-10 minutes) for G1, in contrast to 26.52±11.22 minutes (range: 5-44 minutes) for G2 (P=0.00001). Furthermore, a higher compliance with behavioral or pharmacological indications was observed in the G1 group, exhibiting lower adherence in only 12 out of 52 cases compared to 14 out of 25 cases in G2 (P=0.0091). CONCLUSIONS: Telemedicine is a proper solution and an effective tool to manage the therapeutic follow-up of NE and LUTS, ensuring suitable quality standards and reducing social costs, such as the loss of working days and transport costs. The implementation and complete integration of its use into the healthcare system should be the goals to pursue in order to take full advantage of all its potentials.

A schedule for tapering glucocorticoid treatment in patients with severe SARS-CoV 2 infection can prevent acute adrenal insufficiency in the geriatric population.

OBJECTIVE AND DESIGN: Glucocorticoids (GCs) have been widely used in symptomatic patients for the treatment of COVID-19. The risk for adrenal insufficiency must be considered after GC withdrawal given that it is a life-threatening condition if left unrecognized and untreated. Our study aimed to diagnose adrenal insufficiency early on through a GC reduction schedule in patients with COVID-19 infection. PATIENTS AND MEASUREMENTS: From November 2021 to May 2022, 233 patients were admitted to the Geriatric Division of the University Hospital of Padova with COVID-19 infection. A total of 122 patients were treated with dexamethasone, after which the GC tapering was performed according to a structured schedule. It consists of step-by-step GC tapering with prednisone, from 25 mg to 2.5 mg over 2 weeks. Morning serum sodium, potassium, and cortisol levels were assessed 3 days after the last dose of prednisone. RESULTS: At the end of GC withdrawal, no adrenal crisis or signs/symptoms of acute adrenal insufficiency were reported. Median serum cortisol, sodium, and potassium levels after GC discontinuation were, respectively, 427 nmol/L, 140 nmol/L, and 4 nmol/L (interquartile range 395-479, 138-142, and 3.7-4.3). A morning serum cortisol level below the selected threshold of 270 nmol/L was observed in two asymptomatic cases (respectively, 173 and 239 nmol/L, reference range 138-690 nmol/L). Mild hyponatremia (serum sodium 132 to 134 nmol/L, reference range 135-145 nmol/L) was detected in five patients, without being related to cortisol levels. CONCLUSIONS: A structured schedule for the tapering of GC treatment used in patients with severe COVID-19 can reduce the risk of adrenal crisis and acute adrenal insufficiency.

JJ stent dislodgement in the distal ureter: how to recover it in preschool children?

Double-J ureteral stents are usually placed after various urological procedures. The dislodgement of their distal ringlet is a rare complication, whose retrieval is arduous in younger children, due to the small ureteral caliber. We propose our innovative endoscopic approach to recover the dislodged JJ stent. Under 8-9.8 Ch cystoscopy, the ureteral meatus is gently cannulated with a 00.18″ guidewire, then a balloon catheter Passeo 18 3-4 mm (Biotronik, Lake Oswego, OR, USA) is coaxially inserted. A pneumatic dilatation of the vesical-ureteral junction is performed up to 8 atmospheres for 5 minutes under direct vision. Consequently, the ureteral meatus allows the cystoscope passage, and the JJ-stent can be recovered thanks to endoscopic grasping forceps. A mono-J stent is then left in place for 24 hours. Four patients aged 8 months - 4 years have been successfully treated with this approach after that JJ migration was found intraoperatively or during ultrasonography. No intra- or postoperative complications occurred. Postoperative hospital stay was prolonged for one day. During 29.5 medium follow-up no clinical or ultrasonographic signs of vesical-ureteral reflux ensued. Our cystoscopic approach is effective and safe to ensure a prompt endoscopic JJ retrieval without changing neither surgical approach nor the anesthesiological support. We believe that all the pediatric urology centers should know the procedure and have small size balloon catheter available.

Case report: The lesson from opioid withdrawal symptoms mimicking paraganglioma recurrence during opioid deprescribing in cancer pain.

Pain is one of the predominant and troublesome symptoms that burden cancer patients during their whole disease trajectory: adequate pain management is a fundamental component of cancer care. Opioid are the cornerstone of cancer pain relief therapy and their skillful management must be owned by physicians approaching cancer pain patients. In light of the increased survival of cancer patients due to advances in therapy, deprescription should be considered as a part of the opioid prescribing regime, from therapy initiation, dose titration, and changing or adding drugs, to switching or ceasing. In clinical practice, opioid tapering after pain remission could be challenging due to withdrawal symptoms' onset. Animal models and observations in patients with opioid addiction suggested that somatic and motivational symptoms accompanying opioid withdrawal are secondary to the activation of stress-related process (mainly cortisol and catecholamines mediated). In this narrative review, we highlight how the lack of validated guidelines and tools for cancer patients can lead to a lower diagnostic awareness of opioid-related disorders, increasing the risk of developing withdrawal symptoms. We also described an experience-based approach to opioid withdrawal, starting from a case-report of a symptomatic patient with a history of metastatic pheochromocytoma-paraganglioma.

Advanced Adrenocortical Carcinoma: From Symptoms Control to Palliative Care.

The prognosis of patients with advanced adrenocortical carcinoma (ACC) is often poor: in the case of metastatic disease, five-year survival is reduced. Advanced disease is not a non-curable disease and, in referral centers, the multidisciplinary approach is the standard of care: if a shared decision regarding several treatments is available, including the correct timing for the performance of each one, overall survival is increased. However, many patients with advanced ACC experience severe psychological and physical symptoms secondary to the disease and the cancer treatments. These symptoms, combined with existential issues, debase the quality of the remaining life. Recent strong evidence from cancer research supports the early integration of palliative care principles and skills into the advanced cancer patient's trajectory, even when asymptomatic. A patient with ACC risks quickly suffering from symptoms/effects alongside the disease; therefore, early palliative care, in some cases concurrent with oncological treatment (simultaneous care), is suggested. The aims of this paper are to review current, advanced ACC approaches, highlight appropriate forms of ACC symptom management and suggest when and how palliative care can be incorporated into the ACC standard of care.

Short article: Relapsing Whipple's disease: a case report and literature review.

Whipple's disease is a rare infection caused by Tropheryma whipplei, a Gram-negative Bacillus usually found in macrophages of the lamina propria of the small intestine. The typical clinical manifestations of classic Whipple's disease are diarrhea, weight loss, malabsorption, abdominal pain, and arthralgia. The disease's laboratory diagnosis is currently based on duodenal biopsy. Treatment generally includes primary therapy for 2 weeks with intravenous antibiotics capable of reaching high levels in the cerebrospinal fluid, such as ceftriaxone, usually followed by treatment with oral cotrimoxazole for 1 year. Early diagnosis should enable appropriate treatment and improves the prognosis, and prolonged antibiotic treatment often leads to complete remission. Our case report focuses on a 72-year-old man who had been passing watery stools for 1-2 months, accompanied by low-grade fever. He reported profound asthenia, a weight loss of about 3 kg, and loss of appetite. Thirty years earlier (in 1984), he had been working as a horse keeper at a University Department of Agricultural and Veterinary Studies, where he had contracted Whipple's disease. Laboratory tests and microbiological studies led to a diagnosis of recurrent Whipple's disease. Esophagogastroduodenoscopy was performed under deep sedation. Biopsy samples obtained from the stomach and duodenum were stained with hematoxylin and eosin, Giemsa, and periodic acid-Schiff to identify any accumulation of typical periodic acid-Schiff-positive macrophages in the lamina propria. A specific quantitative real-time PCR assay using specific oligonucleotide probes for targeting repeated sequences of Tropheryma whipplei was also performed to detect its DNA in the duodenum samples.

Prevalence of sarcopenia based on different diagnostic criteria using DEXA and appendicular skeletal muscle mass reference values in an Italian population aged 20 to 80.

OBJECTIVE: To identify the mean values and percentiles for ASMM (appendicular skeletal muscle mass) and the prevalence of sarcopenia, in terms of muscle mass reduction, using different cutoffs in a European population. DESIGN: A retrospective analysis on the dataset from a multicenter study on apparently healthy Italian adults conducted between 1999 and 2002. A significant muscle mass loss, necessary to diagnose sarcopenia, was defined in 3 different ways: (1) by subtracting 2 SDs from the mean ASMM index (ASMMI) of a young adult population (20-39 year-olds), as in the Rosetta study and the NHANES survey; (2) by calculating the 15th percentile of the distribution of our young population, corresponding to about 1 SD below the mean ASMMI; (3) by calculating the 20th percentile of the distribution of the ASMMI (as in the Health ABC study) of an elderly population. SETTING: Five centers for the diagnosis and treatment of osteoporosis in various parts of the country (Padova, Verona, Parma, Roma, Napoli). PARTICIPANTS: Participants were 1535 volunteers (1208 women and 327 men) aged 20 to 80 years, drawn from among staff members, university students, lay people contacted by word of mouth, and patients presenting spontaneously for osteoporosis screening. MEASUREMENTS: Body weight and height were measured for all participants. Body composition was assessed by DEXA, and the ASMMI was calculated as the ASMM divided by body height in meters squared. RESULTS: Both men's and women's lean mass in the arms and ASMM were highest in the young group and became lower in older age. In men, the ASMMI dropped gradually from age 20 to 29 to age 60 to 69, then remained stable in the oldest group. In women, the ASMMI gradually increased from age 20 to 29 to age 60 to 69, then dropped among the 70- to 80-year-olds. Based on the 15th percentile of the ASMMI for our young adult reference population, the cutoffs for sarcopenia were 7.59 kg/m(2) in men and 5.47 kg/m(2) in women; if the 20th percentile of the ASMMI in our elderly subjects (>65 years) was considered, the cutoffs were 7.64 kg/m(2) in men and 5.78 kg/m(2) in women. Applying the different diagnostic criteria to the those older than 65, the prevalence of sarcopenia ranged from 0% to about 20% in both genders. CONCLUSION: The 15th percentile (or 1 SD below the mean) of the ASMMI of our young adults, and the 20th percentile of this index for an elderly reference population proved more effective in identifying cases of sarcopenia than subtracting 2 SD from the mean ASMMI of a young adult population.

Dietary intake and physical performance in healthy elderly women: a 3-year follow-up.

BACKGROUND: Aging is generally accompanied by changes in body composition, muscle mass and strength, leading to a decline in motor and functional performance. Physical activity and eating habits could be involved in modulating this paraphysiological deterioration. Aim of our study was to investigate changes in body composition, diet and physical performance in healthy, elderly females over a 3-year follow-up. METHODS: 92 healthy elderly females (70.9±4.0 years) attending a twice-weekly mild fitness program were eligible for the study. They were assessed at baseline and again after 3 years in terms of clinical history, diet, body composition by DEXA, resting energy expenditure, handgrip strength, knee extensor isometric/isotonic strength, and functional performance measured using the Short Physical Performance Battery (SPPB). RESULTS: After 3 years, women had a significant decline in muscle strength (∆ isotonic: -1.4±4.3 kg, ∆ isokinetic: -2.0±6.3 kg, ∆ handgrip: -3.2±5.0 kg; p<0.001) and physical performance (∆ walking time: 0.71±0.9 s, ∆ walking speed: -0.25±0.35 m/s; p<0.001), while their weight and body composition parameters did not change, except for a small decrease in appendicular skeletal muscle mass (-0.4±1.4 kg). There was a significant drop in calorie (∆:-345.7±533.1 kcal/d; p<0.001) and protein intake (∆:-0.14±0.23 g/d; p<0.001), while resting energy expenditure remained stable. ∆ calorie intake correlated with the variation in 4-meter walking time (r: 0.34; p<0.01). CONCLUSIONS: With advancing age, physical performance declines even in healthy, fit females despite a spare of weight and body composition. This decline in physical activity could lead to a lower calorie intake, which would explain why there is no variation in body weight.

Trunk and lower limb fat mass evaluated by dual-energy X-ray absorptiometry in a 20- to 80-year-old healthy Italian population.

BACKGROUND AND AIMS: Global fat mass distribution seems to correlate with different levels of cardio-metabolic risk; centrally distributed fat carries a high risk of cardiovascular disease, while lower limb adiposity may have a protective effect against insulin resistance. Reference data regarding body composition have already been published for the Italian population; the aim of this study was to add reference values for trunk and lower limb fat mass, and their ratio (TLR), developing percentile distributions for age brackets between 20 and 80 years. METHODS: A retrospective analysis of a multicenter, cross-sectional study was conducted and 1,570 healthy Italian adults (1,241 females and 329 males) were selected. The regional fat mass, measured by dual-energy X-ray absorptiometry total body scan, was analyzed and the TLR was calculated. RESULTS: In both genders we observed higher trunk fat mass values in older subjects against a smaller difference in BMI values. The leg fat mass was higher in old men, while it was similar in women at different ages. The TLR values in older subjects doubled those of younger subjects in both genders (62% in males and 71% in females). CONCLUSIONS: The identified ranges for trunk, leg fat mass, and TLR may be used as reference values to describe the global fat mass distribution in healthy individuals and to identify states of altered body fat distribution.

Seek and ye shall find: a case of Q fever in an elderly woman.

This case report concerns an elderly woman referred with a 6-month history of rising and falling fever in the range 38-40°C. She was examined repeatedly by her family doctor and given various antibiotic treatments before being hospitalized in our geriatric unit. Laboratory tests and microbiological studies led to a diagnosis of chronic Q fever, a zoonosis caused by Coxiella burnetii, a Gram-negative obligate intracellular coccobacillus; humans usually become infected by inhaling infectious airborne particles. The diagnosis of Q fever relies on serology with enzyme immunoassay (EIA). The main feature of Q fever is its clinical polymorphism: clinical signs may be aspecific and, in chronic cases, patients often do not recall having had the acute infection. As the most frequent and severe manifestation of Q fever is endocarditis, severe consequences may ensue. In our patient, broad-spectrum antibiotics were given before the serology results were available and rapid clinical improvement was achieved. This unusual disease should therefore also be considered in differential diagnosis of fever in the elderly, and age should not be considered as a contraindication for not performing all studies, because timely and adequate treatment is important partly to preserve elderly patients' self-sufficiency and to prevent them from becoming bedridden.

Changes in healthy elderly women's physical performance: a 3-year follow-up.

BACKGROUND: Inflammatory states, hypovitaminosis D and secondary hyperparathyroidism may have a role in the age-related loss of muscle mass, and physical performance in healthy old people. The aim of this study is to investigate changes in muscle mass, strength and physical performance in healthy, active elderly females over a 3-year follow-up, correlating them with any inflammatory states and PTH and 25-hydroxyvitamin D (25-OHD) levels. METHODS: One hundred healthy females over 65 years of age routinely attending a twice-weekly mild fitness program were eligible for the study. Clinical history, serum parameters, body composition by DEXA, handgrip strength, knee extensor isometric/isotonic strength and functional performance measured using the Short Physical Performance Battery (SPPB) were evaluated at the baseline and after 3 years. RESULTS: After 3 years, the women had a significant decrease in weight (∆:-0.8±3.1 kg; p<0.05) and height (∆:-0.4±0,6 cm; p<0.001), while their BMI and body composition parameters did not change. Only IL-6 (∆: 0.6±2.0; p<0.01) and PTH (∆: 30.7±29.2 ng/L; p<0.001) increased significantly, while there were no changes in 25-OHD levels. There was a significant decrease in all the SPPB results and in muscle strength. ∆ PTH only correlated with the variation in 4-meter walking speed (r: 0.41; p<0.01). CONCLUSIONS: With advancing age, physical performance declines even in healthy, active females despite a spare of muscle mass. The increase in PTH seems to have a role in this decline, that could be clarified by further investigations.