Clinical and radiological results following radial osteotomy in patients with Kienböck's disease: four- to 18-year follow-up

Radial osteotomy is currently advocated for patients with Lichtman's stages II and IIIA of Kienb?ck's disease; its place in the treatment of patients with stage IIIB disease remains controversial. The purpose of this study was to evaluate the medium-term results of this procedure and to compare the outcome in patients with stage IIIB disease and those with earlier stages (II and IIIA). A total of 18 patients (18 osteotomies) were evaluated both clinically and radiologically at a mean follow-up of 10.3 years (4 to 18). Range of movement, grip strength and pain improved significantly in all patients; the functional score (Nakamura Scoring System (NSSK)) was high and self-reported disability (Disabilities of Arm, Shoulder and Hand questionnaire) was low at the final follow-up in all patients evaluated. Patients with stage IIIB disease, however, had a significantly lower grip strength, lower NSSK scores and higher disability than those in less advanced stages. Radiological progression of the disease was not noted in either group, despite the stage. Radial osteotomy seems effective in halting the progression of disease and improving symptoms in stages II, IIIA and IIIB. Patients with less advanced disease should be expected to have better clinical results.     

Analysis of vascular involvement in 460 patients with Behçet's syndrome: Clinical characteristics and associated factors

ObjectivesTo describe demographic and clinical characteristics of vascular involvement in patients with Behçet's syndrome (BS) and to evaluate associations with such involvement.MethodsWe retrospectively evaluated records of 2118 BS patients. In total, 460 patients diagnosed with superficial thrombophlebitis (ST) and/or major vascular events (venous and/or arterial involvements) were included in current analysis. Isolated ST with no accompanying deep venous thrombosis might be accepted as part of skin involvement; therefore, we defined two different outcomes for vascular involvement (“any vascular event” and “major vascular events”) and performed univariable and multivariable logistic regression to assess factors associated with these outcome variables.ResultsOverall, 68 (14.8%) patients had isolated ST, and 392 (85.2%) had major vascular events. The mean age of vascular BS was 33.8 (SD: 10.5) years and median follow-up was 13.9 (Q1–Q3: 8.3–22.9) years. The primary sites of major vascular events were deep venous thrombosis (n = 358, 77.8%), pulmonary arterial involvement (n = 66, 14.3%), extrapulmonary arterial involvement (n = 52, 11.3%), and intracardiac thrombosis (n = 14, 3.0%), respectively. Male sex was significantly associated with a higher risk for both outcome variables. When it was added to analysis, ST itself was the strongest explanatory variable that was associated with major vascular events in all multivariable models (ORs = 11.9, 12.0, 13.0, and 18.9). While HLA-B51 was significantly associated with any vascular event, there was no similar observation for major vascular events.ConclusionMale sex is a well-known risk factor for major vascular events in BS, but our study established that presence of ST was the strongest risk factor.     

Anaesthetic considerations in down’s syndrome: experience with 100 patients and a review of the literature

Down’s Syndrome (Trisomy 21, T21) occurs in approximately 0.15 percent of live births. In addition to the stigmata of the syndrome, other congenital defects are frequently found in these patients. Cardiac lesions are particularly prominent. To determine the complications associated with anaesthesia and surgery we examined the records of 100 consecutive patients (58 males, 42 females) who underwent surgery with general anaesthesia during a two year period, from March 1978-March 1980. In addition to the cardiac lesions, the low birth weight of Trisomy 21 infants, increased suceptibility to infections, atlanto-occipital dislocation, and reduced central nervous system catecholamine levels might be expected to result in an increased incidence of complications. This study of 100 patients with Trisomy 21 (T21) indicates that the incidence of complications is low. However, the anaesthetist must understand the pathophysiology of T21 in order to provide optimal anaesthetic care.     

Beyond 10 years,with or without an intestinal graft: Present and future?

Long‐term outcomes in children undergoing intestinal transplantation remain unclear. Seventy‐one children underwent intestinal transplantation in our center from 1989 to 2007. We report on 10‐year posttransplant outcomes with (group 1, n = 26) and without (group 2, n = 9) a functional graft. Ten‐year patient and graft survival rates were 53% and 36%, respectively. Most patients were studying or working, one third having psychiatric disorders. All patients in group 1 were weaned off parenteral nutrition with mostly normal physical growth and subnormal energy absorption. Graft histology from 15 late biopsies showed minimal abnormality. However, micronutrient deficiencies and fat malabsorption were frequent; biliary complications occurred in 4 patients among the 17 who underwent liver transplantation; median renal clearance was 87 mL/min/1.73 m². Four patients in group 1 experienced late acute rejection. Among the 9 patients in group 2, 4 died after 10 years and 2 developed significant liver fibrosis. Liver transplantation and the use of a 3‐drug regimen including sirolimus or mycophenolate mofetil were associated with improved graft survival. Therefore, intestinal transplantation may enable a satisfactory digestive function in the long term. The prognosis of graft removal without retransplantation is better than expected. Regular monitoring of micronutrients, early psychological assessment, and use of sirolimus are recommended.     

Comparison of postoperative chylothorax in infants and children with trisomy 21 and without dysmorphic syndrome: Is there a difference in clinical outcome?

IntroductionChildren with trisomy 21 are prone to postoperative chylothorax, caused by malformation of the lymphatic system, after cardiac surgery. The clinical course of patients diagnosed with postoperative chylothorax and trisomy 21 was compared to that of patients without dysmorphic syndromes. Additionally, differences between the groups in composition, amount, and duration of chyle were analyzed to better understand chylothorax in patients with trisomy 21.Materials and methodsRetrospective cohort study using inpatient clinical databases during a 10-year period.ResultsA total of 2255 patients underwent cardiac operations during the period, of whom 160 (7.1%) patients were diagnosed with trisomy 21. Chylothorax developed in 122 children; 89 patients were included in our study. Of 160 trisomy 21 patients, 27 (16.9%) developed postoperative chylothorax compared to 62 (3%) of 2095 patients without dysmorphic syndromes (p = < 0.001). Time on ventilation, stay in intensive care, hospital stay, mortality, and composition of chylous effusion did not differ between groups. The rate of thrombosis was significantly lower (p = 0.02) in the trisomy 21 group.ConclusionChildren with trisomy 21 and congenital heart disease are more prone to developing chylothorax after heart surgery than those without dysmorphic syndromes. However if they develop this postoperative complication, mortality, chylous composition, time in ICU, and duration of hospital stay is not different to from that of other infants or children with this complication. This is important information for the medical specialists involved and is helpful in counseling parents of children with trisomy 21 undergoing heart surgery.Level of evidenceThis is a treatment study evidence level III.     

Cyclosporin A in adult patients with Henoch-Schönlein purpura nephritis and nephrotic syndrome; 5 case reports

Henoch-Sch?nlein purpura (HSP) is usually followed by mild renal involvement, but heavy proteinuria may also occur. Limited experience with cyclosporin A in children shows reduction of proteinuria. In this report, the use of cyclosporin A in 5 adult HSP patients with nephrotic-range proteinuria is described. Cyclosporin A in combination with prednisolone was given in 3 patients with HSP nephritis and nephrotic syndrome after a course of other immunosuppressive drugs and in 2 patients as initial treatment. All patients showed complete or partial remission of nephrotic syndrome with cyclosporin A and preserved stable renal function over a follow-up period of 5 years. We conclude that the combination of cyclosporin A with corticosteroids is effective in inducing remission of nephrotic syndrome in adult patients with HSP nephritis.     

Low-grade T-cell lymphoma of the kidney and Waldenström's macroglobulinemia in a patient presenting with renal failure

Renal failure is rarely the presenting manifestation of non-Hodgkin's lymphoma. We describe the unusual case of a patient who presented with uremia due to lymphomatous infiltration of the kidney by a low-grade T-cell lymphoma. The diagnosis of lymphoma was made by renal biopsy. Extrarenal nodular or extra-nodular involvement could not be detected. However, simultaneously, a lymphoplasmacytic lymphoma was found on bone marrow biopsy associated with IgM paraproteinemia. To our knowledge, this is the first report of a renal T-cell lymphoma associated with Waldenstr?m's macroglobulinemia.     

Occurrence of erectile dysfunction, testosterone deficiency syndrome and metabolic syndrome in patients with abdominal obesity. Where is a sufficient level of testosterone?

Aim

The aim of this study was to determine the prevalence of erectile dysfunction (ED), testosterone deficiency syndrome (TDS), and metabolic syndrome in patients with abdominal obesity (AO) and the prevalence of morbidity at different levels of testosterone (TST).

Background

Male sex hormones play an important role in ED and variety of TDS and may have influence on the development of metabolic syndrome. The number of men with AO which constitutes a serious health risk is continuously growing. Currently, there are different views that TST levels are already insufficient, and the patient should benefit from treatment.

Objectives

This study examined the association between ED, testosterone level and metabolic syndrome in men with AO.

Design, setting, and participants

The study was carried out in an outpatient urology center of Urology Clinic and Obesity Center of the Clinic of Internal Medicine. There were 167 participants??men with AO which were examined as part of preventive examination.

Methods

Hormonal, a complete urological and internal evaluation was carried out in every patient.

Results and limitations

We found some degree of ED in 73% (122/167) in men with AO. The TST levels below 14?nmol/l had of these 122 patients 84 patients (68.9%) and 49 patients (40.2%) below 10?nmol/l. In this group of patients, we found 103/167 patients (61.7%) with metabolic syndrome. When we compared TST level and morbidity, we found significantly more patients with diabetes mellitus (DM), hypertension and dyslipidemia in group with TST below 10?nmol/l. We also found difference in the levels of HDL cholesterol and triglycerides in the group of patients with TST 10?C14 and over 14?nmol/l.

Conclusion

Patients over 40?years of age with AO and ED should also be examined for TDS and metabolic syndrome. In this group of patients we found that 113/167 patients (67.6%) had total TST below 14?nmol/l, and sufficient level of TST seems to be above this level.     

Rising PSA in patients with minor LUTS without evidence of prostatic carcinoma: a missing link?

Objectives To determine the role of pressure flowmetry in patients without bothersome lower urinary tract symptoms (LUTS), rising prostate-specific antigen (PSA) levels and diagnosed as having clinical benign prostatic hyperplasia (BPH) after negative (multiple) extended multi-site biopsy. Methods The study enrolled patients with minor LUTS who were referred to our urological practice by their general practitioner because of a rising PSA level (≥4 ng/ml). After exclusion of clinical prostatic carcinoma by digital rectal examination and transrectal ultrasound, all patients underwent at least one set of extended multi-site biopsies to exclude T1c prostate cancer. Patients with negative biopsies (clinical BPH) were subjected to pressure flowmetry whereafter those with bladder outlet obstruction underwent TURP. Results The study included 82 patients, with a mean age of 64.8 years (50.2–78.2 years), satisfying the inclusion criteria. Urodynamic analysis showed that all patients had bladder outlet obstruction. After TURP, eight patients (9.8%) were diagnosed as having histologically proven prostate cancer; 74 patients (90.2%) were diagnosed as having BPH. Patients of the BPH group had a mean preoperative PSA level of 8.8 ng/ml (4.3–25.8 ng/ml) and a mean international prostate symptom score of 8.8 (2–18). The mean detrusor pressure at maximum flow in BPH patients was 89.5 cmH₂O (20–200 cmH₂O). Conclusions An increased PSA in patients with minor or no LUTS, clinical BPH and negative extended multi-site prostate biopsy is strongly correlated to bladder outlet obstruction. Therefore, patients with these characteristics should be treated with TURP.     

Posterior spinal fusion in patients with Ehlers–Danlos syndrome: a report of six cases

Purpose  

There is a paucity of literature describing posterior spinal fusion (PSF) in the Ehlers–Danlos syndrome (EDS) patient. The vast majority of these studies diagnosed EDS clinically. The purpose of this study is to discuss the management and complications of EDS patients with scoliosis treated with PSF at a single institution.     

Clinical and radiological outcomes after conservative treatment of TB spondylitis: is the 15 years’ follow-up in the MRC study long enough?

Introduction

Tuberculosis of the spine is a still a common disease entity, not only in developing countries but is also returning in developed countries especially in the immune-compromised patients. Conservative treatment with chemotherapy is still the main stay of treatment. This article focuses on the clinical and radiological outcomes, and problems with conservative treatment.

Method

The available literature of anti-tuberculosis chemotherapy in managing spinal tuberculosis was reviewed. Data sources included relevant literature of the English language identified through Medline search from 1946 to 2011. Personal experience and unpublished reviews from the authors’ institution were also included.

Results

Although majority of patients respond well to anti-tuberculosis chemotherapy, about 15 % of them develop paradoxical response. The Medical Research Council (MRC) studies have shown that for patients without significant neurological deficits, operative and conservative treatment could produce the same clinical outcome at 15 years follow-up. Patients treated operatively with debridement and spinal fusion with strut graft had faster bony fusion and less kyphotic deformity. In contrast, those treated with drugs alone or with simple debridement without fusion may result in disease reactivation, severe kyphosis or late instability, which in turn may lead to late-onset Pott’s paraplegia, back pain, sagittal imbalance and compromised pulmonary function that are difficult or risky to treat.

Conclusion

Recognition of the clinical and radiologic features of these late sequels is important for the management. Prevention of deformity in the early disease has been added to the modern standard of treatment of TB spine.

     

Diagnostic utility of a second minor salivary gland biopsy in patients with suspected Sjögren's syndrome: A retrospective cohort study

ObjectiveTo determine whether repeated minor salivary gland biopsy (MSGB) has a clinical diagnostic utility in patients with suspicion of Sjögren's syndrome (SS).MethodsClinical, biological, pathological data and physician's diagnosis after each MSGB from patients with suspected primary or secondary SS who had benefited from 2 MSGB at Brest University Hospital between January 1st, 1990 and January 14th, 2015, were retrospectively collected. We compared the characteristics of patients with and without first positive MSGB, concordance between the MSGB, and analyzed the modifications of diagnosis after the second MSGB.ResultsNinety-three patients were included, first MSGB was positive for 23 and negative for 70. Patients with first positive MSGB had more often renal involvement (P < 0.05) and hypergammaglobulinemia (P = 0.01), anti-SSA antibodies (P < 0.05) and positive second biopsy with focus score ≥ 1 or Chisholm > 2 (P < 0.01). The mean time between the 2 MSGB was 5.7 ± 4.3 years. The concordance between the results of the 2 biopsies was low (κ = 0.34). MSGB influenced diagnostic's change in 10 cases where the second MSGB was always guided by new specific clinical manifestations.ConclusionWe observed a low concordance between 2 MSGB in patients with suspected pSS in our study. Despite this variability, performing a second MSGB changed the initial diagnosis in only a minority of the patients and was particularly useful when clinical manifestations had deeply evolved.     

VCAM-1, ICAM-1, and E-selectin in IgA nephropathy and Schönlein-Henoch syndrome: differences between tissue expression and serum concentration

The tissue expressions of vascular cell adhesion molecule 1 (VCAM-1), intercellular adhesion molecule 1 (ICAM-1) and endothelial leukocyte adhesion molecule 1 (E-selectin-1) were investigated in biopsy specimens from 28 patients with different stages of IgA nephropathy (IgAN) and 20 patients with acute renal failure (ARF) or chronic renal diseases (amyloidosis, Alport's glomerulopathy) by immunohistochemistry. The results were compared with the serum levels of the three adhesion molecules. VCAM-1 expression was significantly increased on parietal/tubular epithelial cells in IgAN and ARF. Significantly elevated circulating VCAM-1 levels were measured in IgAN and amyloidosis, but did not correlate with renal function (creatinine clearance). Significantly increased glomerular endothelial/epithelial ICAM-1 expression was found in IgAN and ARF. Intense mesangial ICAM-1 expression was found in mild stages of IgAN and in Sch?nlein-Henoch syndrome. Circulating ICAM-1 was not significantly elevated in IgAN and different renal diseases. VCAM-1 and ICAM-1 expressions of interstitial infiltrating cells were significantly higher in severe than in mild IgAN and associated with an increased infiltration of inflammatory leukocytes. Patients with IgAN and different renal diseases had decreased mesangial and almost absent interstitial E-selectin expression as compared with controls. The circulating E-selectin levels were significantly elevated in ARF. In conclusion, the tissue expression of adhesion molecules in IgAN reflects a continuous inflammatory renal activity. However, only increased circulating VCAM-1 serum levels correlated significantly with the histological state of renal inflammation and could be used as a disease marker.     

Clinical effect of lumacaftor/ivacaftor in F508del homozygous CF patients with FEV1 ≥ 90% predicted at baseline

ObjectiveThe first available CFTR modulator combination for homozygous F508del patients, lumacaftor/ivacaftor, has not been tested in patients with percentage predicted (pp)FEV₁ > 90 in the phase III trials. The objective of this study is to share real life experience about treatment results in this group.MethodsIn this retrospective observational study, patients aged 6 years or older starting on lumacaftor/ivacaftor in standard care were in strict follow up. For these patients, data were obtained about FEV₁, BMI, CFQ-R and sweat chloride before start and after 6 months of treatment, and data about FEV₁ and BMI were recorded every 3 months. Exacerbations were recorded continuously.ResultsWe identified 40 patients with a ppFEV₁ ≥ 90 at the start of lumacaftor/ivacaftor who had been in follow up for at least 12 months. After 12 months, ppFEV₁ was unchanged, whereas mean absolute change in BMI was +0.88 (p = 0.001) with a mean change in SDS for BMI of +0.26 (p = 0.014). Mean CFQ-R overall score at 6 months improved by 2.6% (p = 0.004) and mean decrease in sweat chloride was -27.3 mEq/L (p = 0.000). Exacerbation rate declined from 1.03 to 0.53/person/year (p = 0.003). One patient discontinued treatment in the first 12 months because of progression of CFRLD, two paused treatment but resumed later.ConclusionHomozygous F508del patients starting lumacaftor/ivacaftor at ppFEV₁ ≥ 90 improved significantly in nutritional status, sweat chloride levels and exacerbation rate, but did not respond in ppFEV₁. Treatment is well tolerated in this patient group. These effects make it worth considering to treat this group of patients with lumacaftor/ivacaftor.     

Clinical and radiological evaluation of Trabecular Metal and the Smith–Robinson technique in anterior cervical fusion for degenerative disease: a prospective, randomized, controlled study with 2-year follow-up

A prospective, randomized, controlled study was carried out to compare the radiological and clinical outcomes after anterior cervical decompression and fusion (ACDF) with Trabecular Metal™ (TM) to the traditional Smith–Robinson (SR) procedure with autograft. The clinical results of cervical fusion with autograft from the iliac crest are typically satisfactory, but implications from the donor site are frequently reported. Alternative materials for cervical body interfusion have shown lower fusion rates. Trabecular Metal is a porous tantalum biomaterial with structure and mechanical properties similar to that of trabecular bone and with proven osteoconductivity. As much as 80 consecutive patients planned for ACDF were randomized for fusion with either TM or tricortical autograft from the iliac crest (SR) after discectomy and decompression. Digitized plain radiographic images of 78 (98%) patients were obtained preoperatively and at 2-year follow-up and were subsequently evaluated by two senior radiologists. Fusion/non-fusion was classified by visual evaluation of the A–P and lateral views in forced flexion/extension of the cervical spine and by measuring the mobility between the fused vertebrae. MRI of 20 TM cases at 2 years was successfully used to assess the decompression of the neural structures, but was not helpful in determining fusion/non-fusion. Pain intensity in the neck, arms and pelvis/hip were rated by patients on a visual analog scale (VAS) and neck function was rated using the Neck Disability Index (NDI) the day before surgery and 4, 12 and 24 months postoperatively. Follow-ups at 12 and 24 months were performed by an unbiased observer, when patients also assessed their global outcome. Fusion rate in the SR group was 92%, and in the TM group 69% (P < 0.05). The accuracy of the measurements was calculated to be 2.4°. Operating time was shorter for fusion with TM compared with autograft; mean times were 100 min (SD 18) and 123 min (SD 23), respectively (P = 0.001). The patients’ global assessments of their neck and arm symptoms 2 years postoperatively for the TM group were rated as 79% much better or better after fusion with TM and 75% using autograft. Pain scores and NDI scores were significantly improved in both groups when compared with baseline at all follow-ups, except for neck pain at 1 year for the TM group. There was no statistically significant difference in clinical outcomes between fusion techniques or between patients who appeared radiologically fused or non-fused. There was no difference in pelvic/hip pain between patients operated on with or without autograft. In our study, Trabecular Metal showed a lower fusion rate than the Smith–Robinson technique with autograft after single-level anterior cervical fusion without plating. There was no difference in clinical outcomes between the groups. The operative time was shorter with Trabecular Metal implants.     

Efficacy,safety, and tolerability of mirabegron in patients aged ≥65 yr with overactive bladder wet: a phase IV,double-blind,randomised, placebo-controlled study (PILLAR)

BackgroundThe majority of patients with overactive bladder (OAB) are aged >65 yr. There has been no prospectively designed study assessing treatment efficacy with the β3-adrenoreceptor agonist, mirabegron, specifically in this age group.ObjectiveA phase IV study comparing flexibly dosed mirabegron versus placebo in elderly patients with OAB and urgency incontinence.Design, setting, and participantsCommunity-dwelling patients aged ≥65 yr with OAB for ≥3 mo.InterventionFollowing a 2-wk placebo run in, patients with one or more incontinence episodes, three or more urgency episodes, and an average of eight or more micturitions/24 h were randomised 1:1 to double-blind 25 mg/d mirabegron or matched placebo, for 12 wk. After week 4 or 8, the dose could be increased to 50 mg/d mirabegron/matched placebo based on patient and investigator discretion.Outcome measurements and statistical analysisCoprimary endpoints: change from baseline to end of treatment (EOT) in the mean numbers of micturitions/24 h and incontinence episodes/24 h. Secondary endpoints: change from baseline to EOT in the mean volume voided/micturition, mean number of urgency episodes/24 h, and mean number of urgency incontinence episodes/24 h. Analysis of covariance (ANCOVA) was used for the mean number of micturitions/24 h, mean volume voided/micturition, and mean number of urgency episodes/24 h. Stratified rank ANCOVA was used for the mean numbers of incontinence episodes/24 h and urgency incontinence episodes/24 h.Results and limitationsStatistically significant improvements were observed for mirabegron versus placebo in change from baseline to EOT in the mean number of micturitions/24 h, mean number of incontinence episodes/24 h, mean volume voided/micturition, mean number of urgency episodes/24 h, and mean number of urgency incontinence episodes/24 h. Safety and tolerability were consistent with the known mirabegron safety profile.ConclusionsMirabegron efficacy, safety, and tolerability over 12 wk were confirmed in patients aged ≥65 yr with OAB and incontinence.Patient summaryWe examined the effect of mirabegron compared with placebo in people aged 65 yr or older with overactive bladder and incontinence. Mirabegron improved the symptoms of overactive bladder compared with placebo. Side effects were similar to those already known for mirabegron.