Congenital intrahepatic portocaval shunts and hypoglycemia due to secondary hyperinsulinism: a case report and review of the literature

Congenital portosystemic shunts present with various associated complications, such as other congenital malformations, hyperammonemia, or hepatopulmonary syndrome. Few cases of associated hypoglycemia have bee...

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An Easy and Sensitive Method to Profile the Antibody Specificities of HLA-Specific Memory B Cells

Background Pre-transplant immunological risk assessment is currently based on donor-specific HLA antibodies in serum. Despite being an excellent source for antibodies produced by bone marrow-residing plasma cells, serum analysis does not provide information on the memory B cell compartment. While B cell culture supernatants can be used to detect memory B cell derived HLA antibodies, low IgG concentrations can preclude detectability of HLA antibodies in luminex single antigen bead (SAB) assays. Method Culture supernatants of polyclonally activated B cells from alloantigen exposed (n=13) or non-exposed (n=10) individuals were either concentrated 10-fold, or IgG was isolated by using a protein G affinity purification method to increase the IgG concentration. These processed culture supernatants, as well as paired serum samples were tested for the presence of HLA antibodies using luminex SAB analysis. Results In immunized individuals, 64% were found to have HLA-specific B cell memory in concentrated supernatants, whereas 82% showed HLA-specific B cell memory when IgG isolated supernatants were used for HLA antibody detection. IgG isolated supernatants showed higher MFI values compared to concentrated supernatants without increased background. In some individuals, HLA-specific B cell memory was detected in the absence of accompanying serum antibody specificities. Conclusions We developed a novel, highly sensitive method to assess the HLA-specific memory B cell compartment using luminex SAB technology. This assay, allows direct comparison to the serum compartment and may therefore provide a more complete picture of the humoral alloimmune response in patients with a history of alloantigen exposure. This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. Corresponding author: Gonca E. Karahan, Department of Immunohematology and Blood Transfusion, Leiden University Medical Center, Albinusdreef 2-2333 ZA Leiden, the Netherlands. e-mail: g.e.karahan@lumc.nl Funding: National Reference Center for Histocompatibility Testing, the Netherlands and the Dutch Transplant Society Astellas Transplantation Research Award 2017. CW was funded by the Swiss National Science Foundation. Conflict of interest: The authors declare no conflicts of interest. Authorship GEK: designed the study, performed experiments, analysed data, wrote manuscript JK/YdV/JLL: performed experiments CW: performed experiments, edited manuscript NML/FJB: provided study samples ItB/MEJR: provided study samples, edited manuscript CvK: designed study, edited manuscript FHJC: interpreted results, edited manuscript SH: designed the study, interpreted results, wrote the manuscript Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.

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A streamlined pathway for patients with unilateral tinnitus: our experience of 22 patients

Abstract

Objectives

Unilateral tinnitus must be investigated with an IAM MRI to rule out a rare but important finding of a cerebello‐pontine angle lesion. A streamlined pathway was designed to uniquely enable GPs to make direct and concurrent referrals for an IAM MRI alongside an audiology‐led one‐stop tinnitus clinic. The primary aim was evaluating the efficacy of the streamlined pathway. The secondary aim was to identify defect measures and associated costs. Design A prospective, experimental non‐randomised control study with retrospective controls was used. Setting Participants were selected from those presenting to the local NHS trust.

Participants

There were 22 adult unilateral tinnitus patients in each of the experimental and control arms, presenting with no red flag or other otological signs/ symptoms or obvious treatable cause for their tinnitus. Main outcome measures Primary endpoints were referral to treatment times, number of patient healthcare visits, number of pathway steps, hand‐off and lead time. Defect measures were ascertained to determine deviation from the intended pathway. The average cost of the new and old pathway were calculated.

Results

The streamlined pathway demonstrated a statistically significant reduction in referral received to treatment, number of patient healthcare visits, number of pathway steps, hand‐off and lead time. 50% of patients seen on the new pathway were redirected from other referrals, 18.2% lacked corresponding IAM MRI request and 54.5% of patients required additional ENT input. The streamlined pathway saved £80 per patient with unilateral tinnitus.

Conclusions

The streamlined unilateral tinnitus patient pathway confers significant benefits to patients, the NHS and wider health economy. Future research should focus on the challenge of scaling and sustaining innovation.

This article is protected by copyright. All rights reserved.

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Temporomandibular Disorders Clinical and Anatomical Outcomes After Fat-Derived Stem Cells Injection

Temporomandibular disorders (TMD) are common disorders that usually involve temporomandibular joint (TMJ), masticatory muscles, and other relevant structures. The symptoms may vary limiting the patients' quality of life. Many treatment options were proposed during the last years with the aim to treat the pathology. In this article, we analyze the effect of the injection of the fat-derived stem cell in the joint as a new treatment option. Address correspondence and reprint requests to Giulia Amodeo, MD, Via G. Martinotti 20, 00135, Rome, Italy; E-mail: gamodeo@live.it Received 31 January, 2018 Accepted 3 July, 2018 The authors report no conflicts of interest. © 2018 by Mutaz B. Habal, MD.

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Technique of The Buccal Fat Pad Flap as an Alternative for the Surgical Defect of Pleomorphic Adenoma

Pleomorphic adenoma (PA) is the most common benign salivary gland neoplasm, and its malignant transformation rarely occurs. Diagnosis is based on clinical features of local or regional malignancy or distant metastasis, and histological identification of invasion and cellular atypia. Buccal fat pad, also called Bichat fat pad, is an excellent option to create tissue coverage over surgically treated areas, providing great blood supply. Thus, this study aims to report a surgical treatment of a patient who reported to the Oral and Maxillofacial Surgery and Traumatology service of a hospital in the city of Fortaleza, presenting a large lesion in the right side of the palate. Clinical examination revealed a nodular volume increase with smooth surface, sessile insertion, firm to palpation and coloration similar to the mucosa, no pain complaints, and 2 years of evolution. Orthopantomographic examination did not reveal alterations in the adjacent anatomical structures. After clinical and radiographic evaluation, an incisional biopsy was performed and histopathological diagnosis was PA. Patient was submitted to surgery for tumor excision with narrow surgical margins, including oral lining mucosa to reduce recurrence chances. In the same surgical act, after the excision of the lesion, the right buccal fat pad pedicle flap was sutured onto the residual mucosa in order to act as a framework for re-epithelialization of the palatal mucosa, phenomenon of metaplasia, and reduction of patient's morbidity. Surgical piece obtained was sent to anatomopathological study, confirming initial diagnosis. After 12-month follow-up, patient is esthetically and functionally rehabilitated, and satisfied with clinical and surgical plans executed. Address correspondence and reprint requests to Jair Queiroz de Oliveira Neto, Resident in Oral and Maxillofacial Surgery, Hospital IJF—Institute Dr. Jose Frota, Fortaleza, Ceara, Brazil; E-mail: Jairqueiroz_neto@hotmail.com Received 29 January, 2018 Accepted 9 July, 2018 The authors report no conflicts of interests. © 2018 by Mutaz B. Habal, MD.

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Rare Median Cleft-Lip Repair With Novel Surgical Procedures Using Bilateral Cleft Margin Flaps

Reports of median cleft lip with nasal anomalies are few, and descriptions of concomitant surgical repair of a median cleft lip and bifid nose deformity are similarly rare. These surgical repair techniques mainly involve the use of normal neighboring structures. Reconstruction of the columella and nasal base is especially important. A new surgical procedure was examined using bilateral cleft margin flaps for patients with a rare median cleft lip. Two patients with a rare median cleft lip were indicated for the procedure. One patient was 3 months old at surgery, and the other patient was 6 months old. These patients underwent single-stage reconstruction of the lip and nose. The new surgical procedure is characterized by the reconstruction of the columella, which involves creating a supportive structure using a cleft margin flap; subsequently, an anterior covering is created using a hinge flap from the nasal tip. Upper lip formation is performed by repair of the orbicularis oris muscle and rotation advancement of a cutaneous and mucosal flap from the lateral segments. The postoperative follow-up period was 4 to 5 years. The postoperative appearance was satisfactory in both patients. Furthermore, no significant complications were observed postoperatively. In conclusion, the surgical procedure with bilateral cleft margin flaps described in this report allows for successful reconstruction of a median cleft lip. This procedure is effectively tissue saving and results in satisfactory outcomes, which are maintained over a long term. Address correspondence and reprint requests to Keisuke Imai, MD, Department of Plastic and Reconstructive Surgery, Osaka City General Hospital, 2-13-22 Miyakojimahondori, Miyakojima-ku, Osaka 534-0021, Japan; E-mail: imai-k@abox3.so-net.ne.jp Received 21 March, 2018 Accepted 9 July, 2018 The authors report no conflicts of interest. © 2018 by Mutaz B. Habal, MD.

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Posterior Distraction First or Fronto-Orbital Advancement First for Severe Syndromic Craniosynostosis

Purpose: Posterior calvarial vault expansion using distraction osteogenesis is performed for syndromic craniosynostosis as the first choice. This procedure allows far greater intracranial volume than fronto-orbital advancement (FOA). This study aimed to determine the most suitable timing of posterior distraction or FOA to sufficiently increase the intracranial volume and remodel the skull shape. Patients and Methods: From 2014 to 2017, the authors performed posterior distraction in 13 patients with syndromic craniosynostosis. Data on premature suture fusion, age at first visit, age at surgery, skull thickness, and complications were collected. Results: Five patients underwent posterior distraction at approximately 12 months of age and had no complications, including cerebrospinal fluid leakage or gull wing deformity. However, during the waiting period for the operation, the skull deformity continues to extend upward (turribrachycephaly). To prevent progress of the skull deformity, the authors performed the operation at approximately 6 months of age in 7 patients. However, in 3 of 7 patients whose lambdoid sutures were opening, gull wing deformity occurred. From these results, in a patient with severe Beare–Stevenson syndrome, the authors performed FOA first at 5 months of age, followed by posterior distraction at 12 months of age, and achieved favorable results. Conclusions: Treatment patterns are patient specific and should be tailored to premature suture fusion, specific skull deformity, and required intracranial volume of each patient. Address correspondence and reprint requests to Yoshiaki Sakamoto, MD, Department of Plastic and Reconstructive Surgery, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ward, Tokyo 160-8582, Japan; E-mail: ysakamoto@z8.keio.jp Received 20 February, 2018 Accepted 9 July, 2018 The authors report no conflicts of interest. © 2018 by Mutaz B. Habal, MD.

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