Management of cervicofacial lymphatic malformations requires a multidisciplinary approach

Background/PurposeCervicofacial lymphatic malformations (CFLM) are rare, potentially life-threatening vascular anomalies, yet reports on multidisciplinary treatment strategies are lacking. We evaluated outcomes for CFLMs following sclerotherapy, surgical resection, and/or medical management.MethodsWe identified children with a CFLM at a vascular anomalies center from 2004 to 2019. Exclusion criteria: retro-orbital malformations, untreated malformations, patients without follow-up. Primary clinical outcome was contour improvement, with significance defined as LM volume reduction of > 50% by cross-sectional imaging.ResultsSixty-three children met inclusion criteria: 35 with macrocystic CFLMs, six with microcystic CFLMs, and 22 with mixed-type malformations. Mean post-intervention follow-up was 27.5 months. Fifty-eight patients underwent sclerotherapy (median: two treatments). Doxycycline and/or bleomycin were used in 95% of patients. After sclerotherapy, 97% of macrocystic CFLMs improved significantly compared to 82% of mixed and 67% of microcystic lesions. Sixteen children underwent surgical resection with 75% significantly improving; two additional patients were successfully treated with sclerotherapy after debulking surgery. Six children received sirolimus for microcystic disease, of which 33% significantly improved.ConclusionSclerotherapy is very effective for macrocystic components of CFLMs, albeit less so for microcystic disease. Microcystic CFLMs frequently require surgical resection. Sirolimus is a helpful therapeutic adjunct, particularly for microcystic lesions, but more study is needed.Level of EvidenceLevel II, prognosis study     

Examining Rules in Friends with Benefits Relationships

Establishing communicative and behavioral boundaries in romantic relationships provides partners with a greater sense of relational stability and certainty. For romantic relationships, these boundaries, such as sexual exclusivity, are relatively straightforward. For casual sex relationships, however, the relational rules are less stable and certain. This exploratory study examined rules in friends with benefits relationships (FWBRs) for 109 college students in the USA. Responses to open-ended questions were collected through an online questionnaire, and data were qualitatively analyzed through an inductive thematic analysis. The data were structured into communication rules, sexual rules, and relational definition rules. Results provide overlap and extension of previous work investigating rules in FWBRs. Notably, participants reported sexual exclusivity as an important rule. Additionally, potentially competing discourses in FWBR rules were best understood through the lens of relational dialectics. Findings reflect a tension in terms of relational work, as partners struggle with maintaining their sexual and friendship relationship while not falling into the “territory” of romantic relationships.

     

Human-Type Blood Factors in Gibbons, with Special Reference to the Multiplicity of Serological Specificities of Human Type M Blood

The human-type blood groups of 20 gibbons of subspecies Hylobates lar lar and of four gibbons of subspecies Hylobates lar pileatus have been determined. Differences in distribution of the ABO blood groups and MN types were observed for the two subspecies, but all 24 gibbons were of Rh-Hr type Rh^Gi. All the animals also lack both factors I and i. By absorption experiments anti-M reagents were shown to contain antibodies of at least three distinct M specificities; one M specificity is shared by all gibbon red cells, a second is absent from all gibbon red cells, while a third is shared only by gibbons red cells of type (M)^Gi and type (MN)^Gi. The theoretical and practical significance of the multiplicity of M factors in human M blood are pointed out.     

IVF - how much more does it cost?

The high cost of some newer drugs will have to be justified by significant clinical improvements. However, not all new drugs are more expensive than older options. These are just some of the findings from a cost analysis of antifungal therapy presented at the International Summit on Cutaneous Antifungal Therapy held in San Francisco late last year. Although preliminary, it has provided a first attempt at comparing costs in a therapeutic area practically devoid of published pharmacoeconomic studies.     

Renal Ultrasound Changes After Pyeloplasty in Children With Ureteropelvic Junction Obstruction: Long-term Outcome in 47 Renal Units

Purpose

We evaluated the use of renal ultrasound for monitoring pyelocaliectasis after pyeloplasty in children.

Materials and Methods

Changes in pyelocaliceal dilatation following pyeloplasty were assessed by serial ultrasound. Of 104 children 0 to 12 years old who underwent pyeloplasty between 1982 and 1992, 44 (47 renal units) were monitored with serial ultrasound for at least 2 years (range 2 to 9, mean 3.8). Patient ages at pyeloplasty were 0 to 3 months (17), 4 to 12 months (8), 1 to 6 years (13) and 7 to 12 years (6). Preoperative and postoperative ultrasound was reviewed by a single pediatric radiologist blinded to the date of surgery. The degree of pyelocaliectasis was graded as 0 to 4 according to the classification of the Society for Fetal Urology.

Results

Preoperative ultrasound revealed grade 4 pyelocaliectasis in 26 kidneys (55 percent) and grade 3 disease in 21 (45 percent). Grade was the same or worse 1 month after pyeloplasty in the majority of kidneys (92 percent) studied at this interval. Of the 47 renal units assessed 43 (91 percent) showed improvement in pyelocaliectasis during postoperative followup. Only 38 percent of the kidneys improved during the first 6 months of followup, while 81 percent were improved 2 years postoperatively. Improvement to grade 0 or 1 dilatation occurred in only 9 kidneys (19 percent). The rate of resolution of pyelocaliectasis was not related to preoperative grade or patient age at pyeloplasty.

Conclusions

Improvement on renal ultrasound after pyeloplasty appears to be gradual. Less than half of the patients had improvement in the initial 6 months after pyeloplasty and pyelocaliectasis rarely resolved completely. While renal ultrasound can provide an accurate and cost-effective means of monitoring children on a long-term basis after pyeloplasty, sonographic evaluation in the early postoperative period commonly shows increased or unchanged pyelocaliectasis.     

Safety of celecoxib in individuals allergic to sulfonamide: a pilot study.

OBJECTIVE: To evaluate cross reactivity between sulfonamide antimicrobials and celecoxib in patients with histories of allergies to sulfonamide antimicrobials. METHODS: Immunocompetent patients with a history of sulfonamide antimicrobial allergy who were being considered for therapy with celecoxib were prospectively enrolled. Sulfamethoxazole and trimethoprim skin prick and intradermal testing and/or an in vitro lymphocyte toxicity assay were performed. If skin testing was negative, an oral challenge with sulfamethoxazole and trimethoprim was performed. Oral challenges with celecoxib were administered to all patients. RESULTS: Twenty-eight immunocompetent patients (26 female; mean age 60 years) were evaluated. History of sulfonamide antimicrobial allergy included urticaria (n = 7), cutaneous eruptions (n = 9), and other (n = 12). Four of the 28 patients who were skin prick tested were positive to sulfamethoxazole and two of the ten patients who underwent in vitro testing were positive to sulfamethoxazole. All 28 patients were administered celecoxib and tolerated the medication. Phone call follow up in 25 patients disclosed that 15 patients continued to take celecoxib, while five patients did not take celecoxib following the oral challenge, and five discontinued celecoxib due to adverse effects, lack of drug efficacy or physician preference. CONCLUSIONS: Confusion exists regarding the potential for cross reactivity between sulfonamide antimicrobials and other sulfonamide-containing compounds. The six sulfonamide-allergic patients tolerated celecoxib uneventfully. This pilot study supports the hypothesis that the potential for cross-reactivity between celecoxib and sulfonamide antimicrobials appears to be low. However, further investigations are required to confirm this.     

Quantitation of motexafin lutetium in human plasma by liquid chromatography-tandem mass spectrometry and inductively coupled plasma-atomic emission spectroscopy

Liquid chromatography-tandem mass spectrometry (LC-MS/MS) and inductively coupled plasma-atomic emission spectroscopy (ICP-AES) methods were developed and validated for the evaluation of motexafin lutetium (MLu, lutetium texaphyrin, PCI-0123) pharmacokinetics in human plasma. The LC-MS/MS method was specific for MLu, whereas the ICP-AES method measured total elemental lutetium. Both methods were fast, simple, precise, and accurate. For the LC-MS/MS method, a closely related analogue (PCI-0353) was used as the internal standard (IS). MLu and the IS were extracted from plasma by protein precipitation and injected onto and LC-MS/MS system configured with a C18 column and an electrospray interface. The lower limit of quantitation was 0.05 μg MLu mL⁻¹, with a signal-to-noise ratio of 15∶1. The response was linear from 0.05 to 5.0 μg MLu mL⁻¹. For the ICP-AES method, indium was used as the IS. The sample was digested with nitric acid, diluted, filtered, and then injected onto the ICP-AES system. Two standard curve ranges were validated to meet the expected range of sample concentrations: 0.5 to 50, and 0.1 to 10 μg Lu mL⁻¹. The LC-MS/MS and ICP-AES methods were validated to establish accuracy, precision, analyte stability, and assay robustness. Interday precision and accuracy of quality control samples were ≤6.3% coefficient of variation (CV) and within 2.2% relative error (RE) for the LC-MS/MS method, and ≤8.7% CV and within 4.9% RE for the ICP-AES method. Plasma samples from a subset of patients in a clinical study were analyzed using both methods. For a representative patient, over 90% of the elemental lutetium in plasma could be ascribed to intact MLu at early time points. This percentage decreased to 59% at 48 hours after dosing, suggesting that some degradation and/or metabolism of the drug may have occurred.