Folliculotropic mycosis fungoides driven by DOCK8 immunodeficiency syndrome

DOCK8 immunodeficiency syndrome (DIDS) represents a rare primary immunodeficiency associated with cutaneous viral infections, allergy, and increased risk of malignancy. We report a case of folliculotropic mycosis fungoides with spontaneous resolution occurring in a patient with DIDS.     

Endoscopic removal of foreign bodies:A retrospective study in Japan

BACKGROUND The ingestion of foreign bodies(FBs) and food bolus impaction(FBI) in the digestive tract are commonly encountered clinical problems.Methods to handle such problems continue to evolve offering advantages,such as the avoidance of surgery,reduced cost,improved visualization,reduced morbidity,and high removal success rate.However,to date,no studies have evaluated the endoscopic management of FBs in Japan.AIM To elucidate level of safety and efficacy in the endoscopic management of FBs and FBI.METHODS A total of 215 procedures were performed at Keio University Hospital between November 2007 and August 2018.Data were collected from medical charts,and endoscopic details were collected from an endoscopic reporting system.Procedures performed with a flexible gastrointestinal endoscope were only taken into account.Patients who underwent a technique involving FB or FBI from the digestive tract were only included.Data on patient sex,patient age,outpatient,inpatient,FB type,FB location,procedure time,procedure type,removal device type,success,and technical complications were reviewed and analyzed retrospectively.RESULTS Among the 215 procedures,136(63.3%) were performed in old adults(≥ 60 years),180(83.7%) procedures were performed in outpatients.The most common type of FBs were press-through-pack(PTP) medications [72(33.5%) cases],FBI[47(21.9%)],Anisakis parasite(AP) [41(19.1%) cases].Most FBs were located in the esophagus [130(60.5%) cases] followed by the stomach [68(31.6%) cases].AP was commonly found in the stomach [39(57.4%) cases],and it was removed using biopsy forceps in 97.5% of the cases.The most common FBs according to anatomical location were PTP medications(40%) and dental prostheses(DP)(40%) in the laryngopharynx,PTP(48.5%) in the esophagus,AP(57.4%) in the stomach,DP(37.5%) in the small intestine and video capsule endoscopy device(75%) in the colon.A transparent cap with grasping forceps was the most commonly used device [82(38.1%) cases].The success rate of the procedure was100%,and complication were observed in only one case(0.5%).CONCLUSION Endoscopic management of FBs and FBI in our Hospital is extremely safe and effective.     

A radial head prosthesis that aligns with the forearm axis of rotation: a retrospective multicenter study

BackgroundAdvancements in technique and devices and a better understanding of forearm kinematics have contributed to the improvement in outcomes following radial head arthroplasty. The results of radial head arthroplasty depend on the inherent variability of the proximal radial anatomy and on the subsequent difficulties in attaining anatomic positioning of the prosthesis. A monobloc prosthesis with an anatomical head that is aligned with the patient’s axis of forearm rotation has been recently introduced. We report the clinical and radiographic outcomes for patients across multiple centers who received this radial head implant with a minimum of 2-year follow-up.MethodsThis was a multi-institution retrospective follow-up including 6 centers and multiple surgeons. A total of 114 cases were performed prior to July 20, 2019, of which 52 (46%) responded to request for follow-up. Data collected included Mayo Elbow Performance Score, Disabilities of the Arm, Shoulder and Hand scores, visual analog scale (VAS) for pain, major complications, and reoperations. Major complications were defined as any prosthesis-related event which may or may not have required reoperation.ResultsThe mean Mayo Elbow Performance Score was 87.0 (range, 55-100), with 46% (24/52) scoring a 100. The mean VAS for pain at rest was 0.9 (range, 0-10), and the mean VAS for pain while unscrewing a bottle cap was 1.9 (range, 0-10). The mean Disabilities of the Arm, Shoulder and Hand score was 19.6 (range, 0-88.6). We recorded a total of 4 major complications, 3 of which required reoperations, for a rate of 7.6% and 5.7%, respectively.DiscussionThe current results, collected from 6 institutions and multiple surgeons, are comparable to those of other similar series for radial head prostheses. Our series had lower rates of complications and reoperations. There were no cases of stem loosening. The alignment of the radial head to the forearm axis of rotation may restore forearm kinematics. This design may lead to radiocapitellar contact pressures that are more physiologic in load transmission.     

Building Blocks for Healthy Children: Evaluation of a Child Care Center–Based Obesity Prevention Pilot Among Low-Income Children

ObjectiveTo assess the impact of a multilevel nutrition intervention for low-income child care environments, staff, and center-enrolled children.DesignA cluster-randomized, controlled trial conducted among eligible centers. Staff and parent self-report surveys and objective field observations at baseline and follow-up were conducted.SettingA total of 22 low-income child care centers (enrolling ≥ 25 2- to 5-year-old children).ParticipantsChildren aged 18–71 months; 408 children and 97 staff were randomized into intervention (208 children and 50 staff) and waitlist-control groups (200 children and 45 staff). Retention rates were high (87% for children and 93% for staff).Intervention(s)A 6-session, 6-month director's child nutrition course with on-site technical support for center teachers.Main Outcome Measure(s)Center nutrition/physical activity environment; staff feeding styles, dietary patterns, and attitudes about food; child food preferences and dietary patterns.AnalysisCovariance regression analyses to assess the intervention effect, adjusting for clustering within centers.ResultsSignificant intervention effects were found for the center nutrition training/education environment (b = 3.01; P = .03), nutrition total scores (b = 1.29; P = .04), and staff-level prompting/encouraging feeding styles (b = 0.38; P = .04). No significant intervention effects were found for child-level measures.Conclusions and ImplicationsCurriculum-driven training and implementation support improved nutritional policies and practices and staff–child interactions during meals. Future research could extend the intervention to families and the evaluation to children's dietary behaviors and weight changes.     

Quantification of acid mucins in the descending colon of rats having simultaneously growing colonic tumors

The acid mucins contained in goblet cells of the descending colon of 34 male Sprague-Dawley rats were histochemically labeled by Alcian blue pH 2.5 and quantified in an image analyzer (Cortex Controller). Twenty-two of these 34 rats were treated with 1,2-dimethylhydrazine (DMH) suspended in EDTA solution as a stabilizing agent and the remaining 12 rats with EDTA only. Of the 22 DMH-treated rats, 11 had concomitantly an adenocarcinoma elsewhere in the colon and the remaining 11 rats had no colonic tumors despite DMH treatment. The results indicated that Alcian blue-positive areas occupied 34.5% of the mucosa of the descending colon in tumor-bearing rats, and 35.2% in non-tumor-bearing DMH-treated rats. For EDTA-treated rats the percentage of mucosa occupied by Alcian blue-positive cells was 48.1%. The difference between DMH-treated rats (with or without tumors) and EDTA-treated rats was significant (p less than 0.001). These results suggest that the decrease of Alcian blue areas is related to the protracted treatment with DMH. Whether the decrease in acid mucins is induced by the carcinogen per se or whether it represents a true biochemical premalignant change at the cytoplasmic level remains to be elucidated.     

Two-compartment, two-sample technique for accurate estimation of effective renal plasma flow: theoretical development and comparison with other methods

Discordance between effective renal plasma flow (ERPF) measurements from radionuclide techniques that use single versus multiple plasma samples was investigated. In particular, the authors determined whether effects of variations in distribution volume (Vd) of iodine-131 iodohippurate on measurement of ERPF could be ignored, an assumption implicit in the single-sample technique. The influence of Vd on ERPF was found to be significant, a factor indicating an important and previously unappreciated source of error in the single-sample technique. Therefore, a new two-compartment, two-plasma-sample technique was developed on the basis of the observations that while variations in Vd occur from patient to patient, the relationship between intravascular and extravascular components of Vd and the rate of iodohippurate exchange between the components are stable throughout a wide range of physiologic and pathologic conditions. The new technique was applied in a series of 30 studies in 19 patients. Results were compared with those achieved with the reference, single-sample, and slope-intercept techniques. The new two-compartment, two-sample technique yielded estimates of ERPF that more closely agreed with the reference multiple-sample method than either the single-sample or slope-intercept techniques.