Combined Percutaneous Transhepatic Lymphatic Embolization and Peroral Duodenal Mucosal Radiofrequency Ablation to Manage Protein-Losing Enteropathy.

Percutaneous transhepatic lymphatic embolization (PTLE) and peroral esophagogastroduodenoscopy (EGD) duodenal mucosal radiofrequency ablation (RFA) were performed to manage protein-losing enteropathy (PLE) in patients with congenital heart disease (CHD). Five procedures were performed in 4 patients (M/F = 3/1, median age: 49 years [range 31-71 years]). Transhepatic lymphangiography demonstrated abnormal peri-duodenal lymphatic channels. After methylene blue injection through transhepatic access, subsequent EGD evaluation showed methylene blue extravasation at various sites in the duodenal mucosa. Endoscopic RFA of the leakage sites followed by PTLE using 3:1 ethiodized oil to n-butyl cyanoacrylate glue resulted in improved symptoms and serum albumin (pre-procedure: 2.6 g/dL ± 0.2; post-procedure: 3.5 g/dL ± 0.4, p=0.004) over a median follow-up of 16 months (range 5-20). Transhepatic lymphangiography and methylene blue injection with EGD evaluation of the duodenal mucosa can help diagnose PLE. Combined PTLE and EGD-RFA can be considered to treat patients with PLE.

Nonparasitic Splenic Cysts in Children: Outcomes after Sclerotherapy or Surgical Resection as Initial Management.

Management of splenic cysts in children remains undefined. Sclerotherapy is an innovative, less invasive treatment. This study examined the safety and preliminary effectiveness of sclerotherapy for splenic cysts in children compared with those of surgical treatment. A retrospective review of pediatric patients treated for nonparasitic splenic cysts from 2007 to 2021 was performed at a single institution. Posttreatment outcomes for patients who underwent either expectant management, sclerotherapy, or surgery were reviewed. Thirty patients aged between 0 and 18 years met the inclusion criteria. Cysts in 3 of 8 patients who underwent sclerotherapy were either unresolved or recurred. Patients who underwent sclerotherapy and required surgery for residual symptomatic cyst had an initial cyst diameter of >8 cm. Symptoms resolved in 5 of 8 patients who underwent sclerotherapy, with a significantly reduced cyst size compared with that in patients with continued symptoms who underwent sclerotherapy (61.4% vs 7.0%, P = .01). Sclerotherapy is an effective treatment for splenic cysts, particularly those measuring <8 cm. However, surgical excision may be preferable for large cysts.

In patients with acute HF and iron deficiency, IV ferric carboxymaltose improved quality of life at 4 to 24 wk.

SOURCE CITATION: Jankowska EA, Kirwan BA, Kosiborod M, et al. The effect of intravenous ferric carboxymaltose on health-related quality of life in iron-deficient patients with acute heart failure: the results of the AFFIRM-AHF study. Eur Heart J. 2021;42:3011-20. 34080008.

Weight gain and resource utilization in infants after fundoplication versus gastrojejunostomy.

PURPOSE: There is wide practice variation in the use of laparoscopic fundoplication (LF) versus gastrojejunostomy (GJ) tube insertion for children who do not tolerate gastric feeds. Using weight gain as an objective proxy of adequate nutrition, we sought to evaluate the difference in weight gain between LF and GJ. METHODS: A retrospective, cohort study was conducted of patients ≤ 2 years who underwent LF or GJ between 2014 and 2019 at a single institution. Patient characteristics, change in weight 1-year post-procedure and frequency of unplanned healthcare utilization encounters were collected and examined. RESULTS: A total of 125 patients (50.4%LF, 49.6%GJ) were identified. Adjusted modeling demonstrated that on average, there was an additional 0.85-unit increase in weight-for-age Z scores in the LF compared to the GJ cohort (p = 0.01). The GJ cohort had significantly more unplanned healthcare utilization encounters (4.2, SD 3.4) compared to LF (3.0, SD 3.1) (p = 0.03). Furthermore, the GJ cohort underwent an average of 3.3 planned GJ exchanges within 1-year post-procedure. CONCLUSION: In the first year post-operatively, LF is associated with increased weight gain and fewer unplanned and overall healthcare encounters compared to GJ. Long-term outcomes including weight gain and quality-of-life measures should be studied to develop standardized guidelines for this common clinical scenario.

Population pharmacokinetics of free flucloxacillin in patients treated with oral flucloxacillin plus probenecid.

Oral flucloxacillin may be coadministered with probenecid to reduce flucloxacillin clearance and increase attainment of pharmacokinetic-pharmacodynamic (PK/PD) targets. The aims of this study were to develop a population PK model of free flucloxacillin when administered orally with probenecid, and to identify optimal dosing regimens for this combination. METHODS: We performed a prospective observational study of adults (45 participants) treated with oral flucloxacillin 1000 mg and probenecid 500 mg 8-hourly for proven or probable staphylococcal infections. Steady-state mid-dose-interval flucloxacillin measurements (45 concentrations) were combined with existing data from a crossover study of healthy participants receiving flucloxacillin with and without probenecid (11 participants, 363 concentrations). We developed a population pharmacokinetic model of free flucloxacillin concentrations within Monolix, and used Monte Carlo simulation to explore optimal dosing regimens to attain PK/PD targets proposed in the literature (free drug time above minimum inhibitory concentration). RESULTS: Flucloxacillin disposition was best described by a 1-compartment model with a lag time and first-order absorption. Free flucloxacillin clearance depended on probenecid, allometrically-scaled fat free mass (FFM) and estimated glomerular filtration rate (eGFR). Predicted PK/PD target attainment was suboptimal with standard dosing regimens with flucloxacillin alone, but substantially improved in the presence of probenecid. CONCLUSION: The simulation results reported can be used to identify dose regimens that optimise flucloxacillin exposure according to eGFR and FFM. Patients with higher FFM and eGFR may require the addition of probenecid and 6-hourly dosing to achieve PK/PD targets. The regimen was well-tolerated, suggesting a potential for further evaluation in controlled clinical trials to establish efficacy.

Midline venous catheters as an alternative to central line catheter placement: a product evaluation.

Midline catheters have emerged as a successful alternative to peripheral venous catheters (PVCs) and central venous catheters (CVCs) in select patients. Midline catheters allow for greater duration of access when compared with PVCs and avoid the critical complications associated with CVCs. This article describes the implementation of the Powerwand® midline at a large paediatric tertiary care facility in acute and intensive care settings, and presents illustrative cases of the catheter in use. Product evaluation was performed by a paediatric vascular access team of registered nurses and included information on patient outcomes, inserter satisfaction, patient satisfaction, and cost effectiveness.

Surveillance of Leprosy in Kiribati, 1935-2017.

In Kiribati, unlike most countries, high and increasing numbers of cases of leprosy have been reported despite the availability of multidrug therapy and efforts to improve case finding and management. Historic records show that 28 cases had been identified by 1925. A systematic population survey in 1997 identified 135 new cases; the mean incidence rate for 1993-1997 was 7.4/10,000 population. After administering mass chemoprophylaxis, the country reached the elimination threshold (prevalence <1/10,000), but case numbers have rebounded. The mean annualized rate of new cases in 2013-2017 was 15/10,000 population, with the highest new case rates (>20/10,000 population) in the main population centers of South Tarawa and Betio. Spread is expected to continue in areas where crowding and poor socioeconomic conditions persist and may accelerate as sea levels rise from climate change. New initiatives to improve social conditions are needed, and efforts such as postexposure chemoprophylaxis should be implemented to prevent spread.

Abernethy Malformations: Evaluation and Management of Congenital Portosystemic Shunts.

PURPOSE: To assess the utility of preoperative venography in evaluating and managing patients with congenital portosystemic shunts (CPSSs). MATERIALS AND METHODS: A retrospective study was performed of 42 patients (62% female; median age, 4.1 years) diagnosed with a CPSS from 2005 to 2018. Preoperative venography (n = 39) and balloon occlusive pressure measurements (n = 33) within the mesenteric venous system guided treatment. Primary outcome was serum ammonia levels at 1 month after shunt closure. Management strategies included single (n = 12) or staged (n = 18) operative ligation, endovascular occlusion (n = 8), combined surgical and endovascular closure (n = 2), and observation (n = 2). RESULTS: At 1 month, serum ammonia levels decreased from 82.5 ± 10.3 µmol/L to 38.4 ± 4.6 µmol/L (P < .001). No difference was observed in the decrease between patients treated surgically or endovascularly (P = .91). Mean occluded to non-occluded pressure gradients were significantly lower for endovascular closure (5.3 ± 1.8 mmHg) than for surgical closure (12.3 ± 3.3 mmHg, P = .02). Shunts were classified as extrahepatic in 29 patients and as intrahepatic in 13 patients; all shunts demonstrated filling of the portal system with occlusive venography. Broad and short shunts were closed surgically; narrow and long shunts were closed endovascularly. Shunts were closed in a single session (n = 20) if the pressure gradient was less than 10 mmHg and the occluded mesenteric pressure was less than 25 mmHg. CONCLUSIONS: Preoperative venography delineates shunt morphology, and balloon occlusion simulates closure hemodynamics. This information is necessary to determine whether definitive closure should be performed through endovascular or surgical methods and whether closure should be performed in a single or staged setting.

Deep Vein Thrombosis Due to Fecal Impaction.

BACKGROUND: Deep vein thrombosis (DVT) can occasionally be secondary to mechanical compressions, such as in May-Thurner syndrome. To our knowledge, no cases of DVT associated with mechanical compression by fecal impaction in a pediatric patient have been previously reported. CASE PRESENTATION: An 18-year-old developmentally delayed female presented to the emergency department with swelling involving her left lower extremity. Cross-sectional imaging revealed a significant stool burden in the rectosigmoid colon compressing the left external iliac vein and causing secondary thrombosis. Thrombectomy successfully alleviated the clot burden. CONCLUSION: This case underscores the potentially serious implications of severe fecal impaction in the pediatric and adolescent populations.

Inbreeding removes sex differences in lifespan in a population of Drosophila melanogaster.

Sex differences in ageing rates and lifespan are common in nature, and an enduring puzzle for evolutionary biology. One possibility is that sex-specific mortality rates may result from recessive deleterious alleles in 'unguarded' heterogametic X or Z sex chromosomes (the unguarded X hypothesis). Empirical evidence for this is, however, limited. Here, we test a fundamental prediction of the unguarded X hypothesis in Drosophila melanogaster, namely that inbreeding shortens lifespan more in females (the homogametic sex in Drosophila) than in males. To test for additional sex-specific social effects, we studied the lifespan of males and females kept in isolation, in related same-sex groups, and in unrelated same-sex groups. As expected, outbred females outlived outbred males and inbreeding shortened lifespan. However, inbreeding-mediated reductions in lifespan were stronger for females, such that lifespan was similar in inbred females and males. We also show that the social environment, independent of inbreeding, affected male, but not female lifespan. In conjunction with recent studies, the present results suggest that asymmetric inheritance mechanisms may play an important role in the evolution of sex-specific lifespan and that social effects must be considered explicitly when studying these fundamental patterns.

Treatment of Unicameral Bone Cysts Utilizing the Sclerograft™ Technique.

PURPOSE: To evaluate the Sclerograft™ procedure, which is an image-guided, minimally invasive approach of chemical sclerotherapy followed by bone grafting of unicameral bone cysts (UBC). MATERIALS AND METHODS: A retrospective evaluation from August 2018 through August 2023 was performed at a single institution on patients that underwent the Sclerograft™ procedure for UBCs. Radiographic healing was evaluated utilizing the Modified Neer Classification. Two different regenerative grafts, CaSO4-CaPO4 and HA-CaSO4 were utilized. A total of 50 patients were evaluated with 41 patients grafted with CaSO4-CaPO4 and 9 patients grafted with HA-CaSO4. RESULTS: The average age of the patient was 12.1 years with an average radiographic follow-up of 14.5 months. Average cyst size was 5.5 cm in the largest dimension and average cyst volume was 20.2 cc. 42 out of 50 (84%) showed healed cysts (Modified Neer Class 1) on the most recent radiograph or MRI. Recurrences occurred on average at 7.2 months. Activity restrictions were lifted at 3-4.5 months post-procedure. Cyst stratification by size did not show a difference in recurrence rates (p = 0.707). There was no significant difference in recurrence rate between lesions abutting the physis compared to those that were not abutting the physis (p = 0.643). There were no major complications. CONCLUSIONS: The Sclerograft™ procedure is an image-guided approach to treating unicameral bone cysts, utilizing chemical sclerosis and regenerative bone grafting. The radiographic healing of cysts compares favorably to open curettage and grafting as determined utilizing previously published trials.

Melanoma in infants, caused by a gene fusion involving the anaplastic lymphoma kinase (ALK).

We describe the first cases of pediatric melanoma with ALK fusion gene arising within giant congenital melanocytic nevi. Two newborn boys presented with large pigmented nodular plaques and numerous smaller satellite nevi. Additional expansile nodules developed within both nevi and invasive melanomas were diagnosed before 10 months of age in both boys. Oncogenic driver mutations in NRAS and BRAF were absent in both cases. Instead, oncogenic ZEB2::ALK fusion genes were identified in both the nevus and melanoma developing within the nevus. In both cases, tumors were noted by ultrasound in utero, demonstrated significant nodularity at birth, and progressed to melanoma in the first year of life suggesting that congenital nevi with ALK fusion genes may behave more aggressively than those with other mutations. As ALK kinase inhibitors are effective against a range of tumors with similar ALK fusion kinases, identifying ALK fusion genes in congenital melanocytic nevi may provide an opportunity for targeted therapy.

Audit of antimicrobial stewardship in medical inpatients in Waikato, New Zealand 2021.

AIMS: Given the threat of rising antimicrobial resistance (AMR), 10 audit standards were selected to audit antimicrobial stewardship (AMS) in secondary care to assess guideline adherence and establish quality improvement initiatives in antimicrobial prescribing. METHODS: Patients were included if they received intravenous (IV) antibiotics across seven medical wards in Waikato or Thames hospitals, New Zealand, in November 2021. Audit standards were defined from the regional antimicrobial prescribing policy and adult antimicrobial guidelines. RESULTS: In total, 205 patients were audited. Microbiological sampling standards were met in 87 of 126 occasions (69.0%). Antimicrobial choices adhered to guidelines in 89 of 163 patients (54.6%), where guidelines were available. Documentation of antimicrobial indications in the medical notes and antimicrobial review at 48 to 72 hours met the standards at over 90%. Only 2 of 13 patients (15.4%) receiving piperacillin/tazobactam or a carbapenem were discussed with Infectious Diseases (ID). Documentation of indications and durations on paper-based medication charts was infrequent, around 12%. Evaluating for health equity, similar results were observed for Maori and non-Maori. CONCLUSIONS: Our audit identified specific areas for AMS quality improvement initiatives. Regular audit should become an essential element of the New Zealand AMS strategy. We believe increased AMS resources are required.

Bedside ultrasound evaluation of tendon injuries.

OBJECTIVE: The primary purpose of this study was to investigate the overall accuracy of bedside extremity tendon ultrasound performed by emergency physicians in the emergency department. We also sought to investigate whether or not bedside tendon ultrasonography can be used to expedite the diagnosis and discharge planning in patients with suspected tendon injuries. METHODS: This was a prospective study conducted at 2 academic level 1 trauma centers. Thirty-four patients were enrolled and underwent a comprehensive physical examination of the injured extremity, followed by a bedside ultrasound evaluation to look for tendon disruption. Results of the tendon ultrasound were compared against the findings seen during wound exploration in the emergency department, wound exploration in the operating room, or results from an extremity magnetic resonance imaging (MRI). RESULTS: There were 6 finger injuries, 11 hand injuries, 6 arm injuries, 6 forearm injuries, and 5 lower extremity injuries. Of the 34 total patients, 4 patients had partial tendon injuries, 9 suffered from 100% tendon laceration or rupture, and 21 had no tendon injury noted on exploration or MRI. Bedside ultrasound had a sensitivity, specificity, and accuracy of 100%, 95%, and 97%, respectively. Physical examination had a sensitivity, specificity, and accuracy of 100%, 76%, and 85%, respectively. Average time to bedside ultrasound was 46.3 minutes compared with 138.6 minutes for wound irrigation and exploration, MRI, or surgery consultation. CONCLUSION: Bedside ultrasound is more sensitive and specific than physical examination for detecting tendon lacerations, and takes less time to perform than traditional wound exploration techniques or MRI.

Rare presentation of BK encephalitis in a child: imaging and pathological findings.

Clinically apparent BK virus infections mostly involve the genitourinary system of immunocompromised patients, particularly those who have undergone renal or bone marrow transplantations. Central nervous system involvement is extremely rare. In this report, we describe the pathologic and MR findings of BK encephalitis in a child. Initial involvement of the brainstem and the cervicomedullary junction was noted that later rapidly progressed to involve other regions of the supratentorial brain parenchyma including the basal ganglia and the thalami as well as the cerebellum.

Intralesional Bleomycin Injection and Skin Hyperpigmentation: A Case Series of a Single-Center Experience With a Standardized Skin-Protective Protocol.

Bleomycin is a cytotoxic antibiotic that has a sclerosing effect on vascular endothelium. Small doses can be injected locally to reduce the size of various vascular malformations. Recognition that targeted intralesional injection carries the potential to cause hyperpigmentation, specifically in areas of skin trauma, and has led our department to implement a bleomycin skin-protective protocol. On review of cases performed following protocol implementation, no patients developed hyperpigmentation attributable to iatrogenic skin trauma. We present our bleomycin skin protection protocol to guide peer institutions in the initiation of similar quality improvement initiatives.

Multi inflammatory syndrome in a 16-year-old male following first dose of m-RNA COVID-19 vaccination.

Multisystem Inflammatory Syndrome (MIS) is an uncommon systemic illness that occurs 4-6 weeks after primary infection with SARS-CoV-2. There are emerging reports of MIS arising following vaccination against SARS-CoV-2. We report a 16-year-old male with a multi system inflammatory condition meeting the case definition for MIS following BTN162b2 mRNA SARS-CoV-2 (Pfizer BioNTech) vaccine with no other identifiable precipitant or evidence of primary infection with SARS-Cov-2.

Pathologic Fracture as Presentation of Generalized Lymphangiomatosis in an Eight-Year-Old: A Case Report and Literature Review.

Pathologic fractures commonly occur secondary to abnormal skeletal physiology in the context of benign or malignant lesions. Rarely, pathologic fractures may occur in the context of a lymphatic abnormality, such as generalized lymphangiomatosis. This rare disorder is characterized by variable presentations in a broad age range of patients. By understanding the effect of widespread lymphatic anomalies on various organ systems, clinicians will be able to make this diagnosis earlier and with more certainty.