Correction of symbrachydactyly: a systematic review of surgical options.

Symbrachydactyly is a rare congenital malformation of the hand characterized by short or even absent fingers with or without syndactyly, mostly unilaterally present. The hand condition can vary from a small hand to only nubbins on the distal forearm. This study aims to systematically review the surgical management options for symbrachydactyly and compare functional and aesthetic outcomes.The review was performed according to the PRISMA guidelines. Literature was systematically assessed searching the Cochrane Library, PubMed, Embase, and PROSPERO databases up to January 1, 2023. Studies were identified using synonyms for 'symbrachydactyly' and 'treatment'. Inclusion criteria were the report of outcomes after surgical treatment of symbrachydactyly in humans. Studies were excluded if they were written in another language than English, German, or French. Case reports, letters to the editor, studies on animals, cadaveric, in vitro studies, biomechanical reports, surgical technique description, and papers discussing traumatic or oncologic cases were excluded.Twenty-four studies published were included with 539 patients (1037 digit corrections). Only one study included and compared two surgical techniques. The quality of the included studies was assessed using the Modified Coleman Methodology Score and ranged from 25 to 47. The range of motion was the main reported outcome and demonstrated modest results in all surgical techniques. The report on aesthetics of the hand was limited in non-vascularized transfers to 2/8 studies and in vascularized transfers to 5/8 studies, both reporting satisfactory results. On average, there was a foot donor site complication rate of 22% in non-vascularized transfers, compared to 2% in vascularized transfers. The hand-related complication rate of 54% was much higher in the vascularized group than in the non-vascularized transfer with 16%.No uniform strategy to surgically improve symbrachydactyly exists. All discussed techniques show limited functional improvement with considerable complication rates, with the vascularized transfer showing relative high hand-related complications and the non-vascularized transfer showing relative high foot-related complications.There were no high-quality studies, and due to a lack of comparing studies, the data could only be analysed qualitatively. Systematic assessment of studies showed insufficient evidence to determine superiority of any procedure to treat symbrachydactyly due to inadequate study designs and comparative studies. This systematic review was registered at the National Institute for Health Research PROSPERO International Prospective Register of Systematic Reviews number: CRD42020153590 and received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.Level of evidenceI.Systematic review registrationPROSPERO CRD42020153590.

Painful nodules and cords in Dupuytren disease.

PURPOSE: The etiology of Dupuytren disease is unclear. Pain is seldom described in the literature. Patients are more often disturbed by impaired extension of the fingers. We recently treated a series of patients who had had painful nodules for more than 1 year, and we therefore decided to investigate them for a possible anatomical correlate. METHODS: Biopsies were taken during surgery from patients with Dupuytren disease and stained to enable detection of neuronal tissue. RESULTS: We treated 17 fingers in 10 patients. Intraoperatively, 10 showed tiny nerve branches passing into or crossing the fibrous bands or nodules. Of 13 biopsies, 6 showed nerve fibers embedded in fibrous tissue, 3 showed perineural or intraneural fibrosis or both, and 3 showed true neuromas. Enlarged Pacinian corpuscles were isolated from 1 sample. All patients were pain free after surgery. CONCLUSIONS: Although Dupuytren disease is generally considered painless, we treated a series of early stage patients with painful disease. Intraoperative inspection and histological examination of tissue samples showed that nerve tissue was involved in all cases. The pain might have been due to local nerve compression by the fibromatosis or the Dupuytren disease itself. We, therefore, suggest that the indication for surgery in Dupuytren disease be extended to painful nodules for more than 1 year, even in the early stages of the disease in the absence of functional deficits, with assessment of tissue samples for histological changes in nerves.

The additional value of CT images interpretation in the differential diagnosis of benign vs. malignant primary bone lesions with 18F-FDG-PET/CT.

OBJECTIVE: To evaluate the value of a dedicated interpretation of the CT images in the differential diagnosis of benign vs. malignant primary bone lesions with 18 fluorodeoxyglucose-positron emission tomography/computed tomography (18F-FDG-PET/CT). MATERIALS AND METHODS: In 50 consecutive patients (21 women, 29 men, mean age 36.9, age range 11-72) with suspected primary bone neoplasm conventional radiographs and 18F-FDG-PET/CT were performed. Differentiation of benign and malignant lesions was separately performed on conventional radiographs, PET alone (PET), and PET/CT with specific evaluation of the CT part. Histology served as the standard of reference in 46 cases, clinical, and imaging follow-up in four cases. RESULTS: According to the standard of reference, conventional 17 lesions were benign and 33 malignant. Sensitivity, specificity, and accuracy in assessment of malignancy was 85%, 65% and 78% for conventional radiographs, 85%, 35% and 68% for PET alone and 91%, 77% and 86% for combined PET/CT. Median SUV(max) was 3.5 for benign lesions (range 1.6-8.0) and 5.7 (range 0.8-41.7) for malignant lesions. In eight patients with bone lesions with high FDG-uptake (SUV(max) >or= 2.5) dedicated CT interpretation led to the correct diagnosis of a benign lesion (three fibrous dysplasias, two osteomyelitis, one aneurysmatic bone cyst, one fibrous cortical defect, 1 phosphaturic mesenchymal tumor). In four patients with lesions with low FDG-uptake (SUV(max) < 2.5) dedicated CT interpretation led to the correct diagnosis of a malignant lesion (three chondrosarcomas and one leiomyosarcoma). Combined PET/CT was significantly more accurate in the differentiation of benign and malignant lesions than PET alone (p = .039). There was no significant difference between PET/CT and conventional radiographs (p = .625). CONCLUSION: Dedicated interpretation of the CT part significantly improved the performance of FDG-PET/CT in differentiation of benign and malignant primary bone lesions compared to PET alone. PET/CT more commonly differentiated benign from malignant primary bone lesions compared with conventional radiographs, but this difference was not significant.

Dipetalogastin, a potent thrombin inhibitor from the blood-sucking insect. Dipetalogaster maximus cDNA cloning, expression and characterization.

A cDNA coding for the thrombin inhibitor dipetalogastin has been isolated from a stomach library of Dipetalogaster maximus, a blood-sucking insect. The open reading frame of the cloned inhibitor cDNA codes for a protein of 344 amino-acid residues. Sequence analysis reveals the existence of three repeated homologous main regions, indicating that the inhibitor consists of three domains. Each domain shows a double-headed structure with an internal sequence homology like rhodniin, the thrombin inhibitor from the blood-sucking insect Rhodnius prolixus. Peptide sequence comparisons of the deduced amino-acid sequence exhibit a high homology of the domains I and II to the natural inhibitor dipetalogastin from the stomach content of D. maximus and to rhodniin, respectively. Significant sequence similarities to Kazal-type inhibitors, like the conserved sequence CGXDXXTYXNXC and several cysteine residues, indicate that the thrombin inhibitor from D. maximus is a further blood-sucking insect which belongs to the Kazal-type family (besides rhodniin). A biologically active recombinant protein corresponding to domain II of the dipetalogastin cDNA was expressed in Escherichia coli. The isolated recombinant dipetalogastin with a molecular mass of 12.91 kDa has proved to be a specific thrombin inhibitor similar to its natural counterpart as well as rhodniin and hirudin. The Ki value of the recombinant dipetalogastin was determined to be 49.3 +/- 22.28 fM.

The geranylgeranyl diphosphate synthase gene of Gibberella fujikuroi: isolation and expression.

The rice pathogen, Gibberella fujikuroi, produces large amounts of gibberellins, a group of natural plant hormones, which induce the superelongation (bakanae) disease of rice. Gibberellins are diterpenoid compounds which are synthesized via the isoprenoid pathway. Here we report the isolation and molecular characterization of the geranylgeranyl diphosphate synthase (ggs) gene from G. fujikuroi. Geranylgeranyl diphosphate synthase is a key enzyme in isoprenoid biosynthesis. Southern blot analysis showed that G. fujikuroi has a single copy of the ggs gene, which is not linked to the farnesyl diphosphate synthase gene. This indicates that the genes of the isoprenoid pathway are not clustered in the fungal genome. The ggs gene is not interrupted by an intron and codes for a polypetide of 418 amino acids. Peptide sequence comparison showed a high degree of similarity to the corresponding Neurospora crassa gene (al-3). However, transcription analyses revealed that the ggs gene, in contrast to the analogous N. crassa gene, is not regulated by blue light. Ammonium and glucose did not affect the transcription of the G. fujikuroi ggs gene, indicating that it is not subject to nitrogen and carbon catabolite repression. The G. fujikuroi gene complements a N. crassa al-3 mutant.