Topical tacrolimus therapy in the management of lower extremity ulcers due to prolidase deficiency

Prolidase deficiency is a rare autosomal recessive disorder characterized by cutaneous ulcers, facial dysmorphism, recurrent infections, and intellectual disability. We report a unique case of a 6‐year‐old boy with prolidase deficiency and Crohn's disease who presented with lower extremity ulcers. Cutaneous ulcers due to prolidase deficiency are historically resistant to treatment, and we report success with the novel use of topical tacrolimus.     

Surviving a stroke in South Africa: outcomes of home-based care in a low-resource rural setting

Background: Little is known of stroke outcomes in low- and middle-income countries with limited formal stroke rehabilitation services and of homebased-stroke services delivered within the primary health care (PHC) context by community health workers (CHWs).

Objectives: To describe and analyze the outcomes of patients with stroke from a rural PHC setting in the Western Cape, South Africa.

Methods: In a longitudinal survey, 93 stroke patients, referred to home and community-based care services (HCBC) between June 2015 and December 2017, were assessed at baseline, one month and three months. Changes in function (Barthel Index (BI)), caregiver strain (Caregiver Strain Index (CSI)), impact of environmental factors and satisfaction with stroke care were measured.

Results: HCBC was delayed, fragmented and brief (median session duration 20 minutes (IQR 15.0–30.0)). Although function improved significantly, dependence remained high: median BI score changed from 40.0 (IQR 15.0–70.0) to 62.5 (IQR 30.0–81.25) (p = .019). A third (33.0% (30/91)) of caregivers initially experienced strain and the median CSI score remained 3.0 (IQR 0.0–7.0) (p = .672). Overall, patient and caregiver satisfaction with HCBC was low with only 46.9% (31/66) of caregivers and 17.4% (12/69) of patients satisfied with all aspects of care. Only 47.6% of assistive product needs were met. Environmental factors negatively impacted on patient function and caregiving.

Conclusions: Clinical practice pathways and referral guidelines should be developed for the HCBC platform. Specific training of CHWs, focusing on how to educate, support and train family caregivers, provide assistive devices and refer to health services is needed.     

Response to chemoradiotherapy and lymph node involvement in locally advanced rectal cancer

AIM: To establish the association between lymph node involvement and the response to neoadjuvant therapy in locally advanced rectal cancer.METHODS: Data of 130 patients with mid and low locally advanced rectal adenocarcinoma treated with neoadjuvant chemoradiation followed by radical surgery over a 5-year period were reviewed. Tumor staging was done by endorectal ultrasound and/or magnetic resonance imaging. Tumor response to neoadjuvant therapy was determined by T-downstaging and tumor regression grading (TRG). Pathologic complete response (pCR) is defined as the absence of tumor cells in the surgical specimen (ypT0N0). The varying degrees TRG were classified according to Mandard’s scoring system. The evaluation of the response is based on the comparison between previous clinico-radiological staging and the results of pathological evaluation. χ² and Spearman’s correlation tests were used for the comparison of variables.RESULTS: Pathologic complete response (pCR, ypT0N0, TRG1) was observed in 19 cases (14.6%), and other 18 (13.8%) had only very few residual malignant cells in the rectal wall (TRG2). T-downstaging was found in 63 (48.5%). Mean lymph node retrieval was 9.4 (range 0-38). In 37 cases (28.5%) more than 12 nodes were identified in the surgical specimen. Preoperative lymph node involvement was seen in 77 patients (59.2%), 71 N1 and 6 N2. Postoperative lymph node involvement was observed in 41 patients (31.5%), 29 N1 and 12 N2, while the remaining 89 were N0 (68.5%). In relation to ypT stage, we found nodal involvement of 9.4% in ypT0-1, 22.2% in ypT2 and 43.7% in ypT3-4. Of the 37 patients considered “responders” to neoadjuvant therapy (TRG1 and 2), there were only 4 N+ (10.8%) and the remainder N0 (89.2%). In the “non responders” group (TRG 3, 4 and 5), 37 cases were N+ (39.8%) and 56 (60.2%) were N0 (P < 0.001).CONCLUSION: Response to neoadjuvant chemoradiation in rectal cancer is associated with lymph node involvement.     

改良Devine治疗小儿隐匿性阴茎158例分析

目的总结改良Devine治疗小儿隐匿性阴茎的效果。方法对158例小儿隐匿性阴茎患者采用改良Devine术式治疗,阴茎皮肤完全脱套后,切除发育异常的纤维肉膜组织,阴茎根部皮肤固定成角。结果 158例患儿术后阴茎均显露良好。随访105例,随访2~38个月,105例患儿阴茎均发育正常,效果满意。结论改良Devine术式是治疗小儿隐匿性阴茎的理想术式。该术式操作简单,术后恢复快,并发症少,治疗效果满意。     

Endodontic treatment of a hypertaurodontic mandibular left second molar in a patient with many taurodonts combined with multiple pulp stones

Taurodontism is a rare embryologic anomaly of teeth, defined by an apical displacement of the furcation of roots and enlarged pulp chambers. Taurodontism has been classified as hypo‐, meso‐ or hypertaurodontism according to the severity of the anomaly. The aim of this case report was to illustrate a clinical case with multiple bilateral taurodonts and to describe the endodontic management of the hypertaurodontic mandibular left second molar with a C‐shaped canal and extensive dental pulp calcifications. A healthy 20‐year‐old male patient was referred for the endodontic treatment of his lower left second molar. Cone beam computed tomography revealed a C‐shaped root canal configuration and several dental pulp calcifications in this tooth. The endodontic treatment was performed in two appointments under an operating dental microscope. A panoramic radiograph, made during the 18 months follow‐up appointment, revealed nine other taurodontic molars, most of them associated with dental pulp calcifications.