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Background and Aims: Despite lipid lowering therapy (LLT), reaching LDL-C targets in patients with familial hypercholesterolemia (FH) remains challenging. Our aim was to determine attainment of LDL-C target levels and reasons for not reaching these in female and male FH patients., Methods: We performed a cross-sectional study of heterozygous FH patients in five hospitals in the Netherlands and Norway. Clinical characteristics and information about LLT, lipid levels and reasons for not being on LDL-C treatment target were retrospectively collected from electronic medical records., Results: We studied 3178 FH patients (53.9% women), median age 48.0 (IQR 34.0-59.9) years. Median LDL-C before treatment and on-treatment was higher in women compared to men (6.2 (IQR 5.1-7.3) and 6.0 (IQR 4.9-7.2) mmol/l (p=0.005) and 3.0 (IQR 2.4-3.8) and 2.8 (IQR 2.3-3.5) mmol/L (p<0.001)), respectively. A minority of women (26.9%) and men (28.9%) reached LDL-C target. In patients with CVD, 17.2% of women and 25.8% of men reached LDL-C target. Women received less often high-intensity statins and ezetimibe. Most common reported reasons for not achieving the LDL-C target were insufficient effect of maximum LLT (women 17.3%, men 24.3%) and side effects (women 15.2%, men 8.6%)., Conclusions: In routine practice, only a minority of women and men with FH achieved their LDL-C treatment target. Extra efforts have to be made to provide FH patients with reliable information on the safety of statins and their long-term effects on CVD risk reduction. If statin treatment is insufficient, alternative lipid lowering therapies such as ezetimibe or PCSK9-inhibitors should be considered., Competing Interests: Declaration of competing competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 The Authors. Published by Elsevier B.V. All rights reserved.)

Background: Painful diabetic peripheral neuropathy (PDPN) is common and difficult to treat with limited treatment options. We assessed the efficacy of frequency rhythmic electromagnetic neural stimulation (FREMS) in patients with PDPN., Methods: An uncontrolled prospective survey of patients with PDPN and pain despite at least two lines of pharmacotherapy. The primary outcome, 50% reduction in pain scores at 1 and/or 3 months post FREMS. FREMS was applied to both legs below the knees using 4 sets of electrodes per leg; the treatment consisted of 10 sessions of 35 min applications given over 14 days. FREMS was repeated every 4 months and patients were followed up for 12 months. Pain was assessed using the neuropathic pain symptom inventory (NPSI) and quality of life (QOL) by the EQ-5D., Results: Out of 336 subjects, 248 patients met the inclusion criteria (56% men), average age and diabetes duration were 65 and 12.6 years respectively. FREMS was associated with a median decrease NPSI of 31% at M1 (range -100;+93%), and a median decrease of -37.5% at M3 (range -100;+250%). The 50% reduction in pain was reached in 80/248 (32.3%) and 87/248 (35.1%) after M1 and M3 respectively. The change in NPSI was accompanied by a decrease in self reported use of opiates of >50%., Conclusion: FREMS treatment was associated with a significant reduction in pain severity over a three months period in patients who did not have adequate response to pharmacotherapy. Randomised (sham)-controlled trials examining the role of FREMS as a treatment for PDPN in non-responders to pharmacotherapy are needed., Competing Interests: Dr Abd Tahrani reports non-financial support from BHR Pharmaceuticals Ltd, during the conduct of the study; grants, personal fees, non-financial support, and other from Novo Nordisk, personal fees from Nestle, grants from Sanofi, personal fees from Gilead, non-financial support from Resmed, outside the submitted work. Dr Abd Tahrani is a Novo Nordisk employee. The authors report no other conflict of interest., (© 2023 Imholz et al.)

Introduction: To evaluate the accuracy of CAD/CAM generated splints in orthognathic surgery by comparing planned versus actual post-operative 3D images., Methods: Specific planning software (SimPlant(®) OMS Standalone 14.0) was used to perform a 3D virtual Le Fort I osteotomy in 10 fresh human cadaver heads. Stereolithographic splints were then generated and used during the surgical procedure to reposition the maxilla according to the planned position. Pre-operative planned and postoperative 3D CT scan images were fused and imported to dedicated software (MATLAB(®)) 7.11.) for calculating the translational and rotational (pitch, roll and yaw) differences between the two 3D images. Geometrical accuracy was estimated using the Root Mean Square Deviations (RMSD) and lower and upper limits of accuracy were computed using the Bland & Altman method, with 95 % confidence intervals around the limits. The accuracy cutoff was set at +/- 2 mm for translational and ≤ 4° for rotational measurements., Results: Overall accuracy between the two 3D images was within the accuracy cutoff for all values except for the antero-posterior positioning of the maxilla (2.17 mm). The translational and rotational differences due to the splint were all within the accuracy cutoff. However, the width of the limits of agreement (range between lower and upper limits) showed that rotational differences could be particularly large., Conclusion: This study demonstrated that maxillary repositioning can be accurately approximated and thus predicted by specific computational planning and CAD/CAM generated splints in orthognathic surgery. Further study should focus on the risk factors for inaccurate prediction.

Introduction: Dental pain may have another origin than teeth. It may be caused by myofascial, neurovascular, cardiac, neurological, sinusal or psychological factors. We will discuss 2 rare cases of patients who presented with a cerebellopontine tumor, who initially manifested with symptoms of dental pain., Observation: The first patient, male, 44 years of age presented to his dentist with toothache (47), which led to its extraction. Five months later, a second painful episode, more characteristic, revealed the presence of a vestibular schwannoma, which was successfully treated and led to the disappearance of the pain. The second case, a 43-year-old female presented to her dentist with toothache (46), which lead the dentist perform a root filling. Two years later, with a 3rd episode of dental pain, more relevant of a trigeminal neuralgia, a epidermoid cyst of the right cerebellopontine angle was identified and successfully treated leading to the disappearance of the pain., Discussion: Cerebellopontine tumors of this type may lead, in exceptional cases to symptoms of dental pain. Therefore, in face of atypical tooth or facial pain, both a detailed medical history and a detailed examination are necessary, in order to investigate any neurological signs and symptoms, before undertaking any non-essential dental treatment, which may be detrimental for the patients., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

The aim of this study was to prospectively evaluate the use of a simultaneous Le Fort I osteotomy for completion of nonreducible Le Fort fractures. We analyzed the clinical and radiological data of 44 patients with Le Fort fractures, 9 of whom presented with a nonreducible type. Seven patients with an incomplete Le Fort I fracture had a contralateral Le Fort I osteotomy, and 2 patients with an incomplete Le Fort III fracture had a true bilateral Le Fort I-type osteotomy. We recorded age and sex, mechanism of injury, level of Le Fort fracture, concomitant mandibular fractures, concomitant maxillomandibular fixation (MMF) and its duration, surgical approach, status of healing, and complications. Follow-ups were at 1 week and 1, 3, 6, and 12 months.All patients recovered their normal pretrauma occlusion without the need for postoperative elastic guidance, except for 1 patient who required light class III traction elastics for 3 weeks to achieve the correct occlusion. None of the patients presented with intraoperative or postoperative complications.The present study has demonstrated that completion of nonreducible Le Fort fractures by Le Fort I osteotomy results in a high rate of success.

Objective: To determine whether patient age is an independent predictor of indication for surgical treatment, realization of surgical procedure, and care management modality., Study Design: We analyzed data of 308 patients with facial fractures. Patients were classified into four age groups: (1) 17 to 40 years old; (2) 41 to 64 years old; (3) 65 to 80 years old; (4) more than 81 years old., Results: Multivariate analysis showed that the patient's age was associated with decreased indication and realization rate for surgery (P = .03 in Group 3; P = .05 in Group 4) and with an increased hospitalization rate (P = .004 in Group 3; P = .02 in Group 4). The presence of concomitant injuries and the fracture's location were associated with a decreased indication and realization rate for surgery regardless age., Conclusion: The current study demonstrated that the patient's age was an independent predictor influencing the treatment decision and realization process as well as the care management modality., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Objective: To evaluate the reliability, accuracy and rapidity of a specific computational method for assessing the orbital floor fracture area on a CT scan., Method: A computer assessment of the area of the fracture, as well as that of the total orbital floor, was determined on CT scans taken from ten patients. The ratio of the fracture's area to the orbital floor area was also calculated. The test-retest precision of measurement calculations was estimated using the Intraclass Correlation Coefficient (ICC) and Dahlberg's formula to assess the agreement across observers and across measures. The time needed for the complete assessment was also evaluated., Results: The Intraclass Correlation Coefficient across observers was 0.92 [0.85;0.96], and the precision of the measures across observers was 4.9%, according to Dahlberg's formula .The mean time needed to make one measurement was 2 min and 39 s (range, 1 min and 32 s to 4 min and 37 s)., Conclusion: This study demonstrated that (1) the area of the orbital floor fracture can be rapidly and reliably assessed by using a specific computer system directly on CT scan images; (2) this method has the potential of being routinely used to standardize the post-traumatic evaluation of orbital fractures., (Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.)

Septic thrombosis of the cavernous sinus (STCS) is an uncommon and potentially lethal disease. Sphenoid and ethmoid sinusitis followed by facial cutaneous infections represents the most common aetiologies, with Staphylococcus aureus as the main responsible organism followed by the Streptococcus pneumoniae. Although all infectious foci of the head and neck area can potentially spread to the cavernous sinus, STCS from oral infection is an exceptionally rare occurrence. We report the unusual case of a patient who presented with an acute STCS secondary to a generalized Streptococcus milleri periodontitis. This case highlights the importance of systematically performing a detailed examination of the oral cavity in patients presenting with intracranial infections caused by uncommon pathogens such as the Streptococcus milleri group.

Purpose: To prospectively assess the incidence of orthoptic anomalies and, more specifically, the possible development of pseudo-Brown syndrome related to inferior oblique muscle (IO) myotomy after the transcaruncular-transconjunctival approach for orbital fracture repair and its effect on the possible development of annoying diplopia., Patients and Methods: We analyzed the clinical data from 14 patients with severe isolated medial wall or combined medial wall/floor fractures treated using titanium meshes placed using a transcaruncular-transconjunctival approach. All patients were assessed with a pre- and postoperative ophthalmologic examination using the following methods: prisms and alternate cover test in all 9 gaze directions, Hess-Weiss coordimetry, Maddox rod screen testing, and Harms wall deviometry. The Bielschowsky head-tilt and a forced duction test were also performed. The patients were classified into the following 2 groups: IO paretic-underaction group and non-IO paretic-underaction group., Results: Of the 14 patients, 7 (50%) fulfilled the criteria for IO paretic-underaction. All 7 developed double vision limited to the extreme upgaze in adduction. Four patients in the non-IO paretic-underaction group had double vision limited to the extreme upgaze in abduction that was already present preoperatively. Diplopia did not interfere with the daily activities in the patients from either group., Conclusions: The present study has demonstrated that IO myotomy associated with the combined transcaruncular-transconjunctival approach can result in orthoptic complications. Moreover, our study has shown that the development of a pseudo-Brown syndrome related to IO underaction was not uncommon but resulted in sequela restricted to a very limited portion of the binocular field of vision., (Copyright © 2012 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.)

Introduction: Maxillary non-union following Le Fort I osteotomy is a rare complication in orthognathic surgery. We report our experience and analyze the possible etiological factors., Patients and Method: We retrospectively analyzed the files of 150 patients having undergone Le Fort I osteotomy, between 1996 and 2006, screening for maxillary non-union. We documented patients' features of: sex, age, medical history, indication, orthodontics, osteotomy type(s) and displacement(s), osteosynthesis, quality of dental occlusion, orthodontics, clinical signs supporting a diagnosis of non-union, radiologic examinations, peroperative observations and surgical revision, outcome after surgical revision., Results: Maxillary non-union was observed in four patients (2.6%). They were female patients with a mean age of 34 years (30 to 38 years) without any specific medical history. All underwent bimaxillary osteotomy (including one Le Fort I segmented osteotomy) for a class III (retromaxillary and promaxillary) associated to vertical disharmony (open-bite and/or transverse discrepancy). Maxillary displacements were always associated to advancement (average: 6mm, 4 to 8mm) and a vertical displacement (upward in three cases, downward in one case). In every case the maxilla was osteosynthesized with titanium miniplates, with four holes and 1.5 screws. Postoperative dental occlusion was deficient in every case, requiring surgical revision of orthodontics between four to six weeks after osteotomy. Clinical signs suggesting non-union were in every case maxillary mobility associated in three cases to discomfort. The mean delay between osteotomy and the non-union was 15.5 months (six to 56 months). These signs appeared after infection in three cases (maxillary sinusitis in two cases, dacryocystitis in one case). Conventional radiological (panoramic and lateral cephalometric radiographs) suggested non-union (plate fracture) in two cases and 3D computed tomography was required in every case to make a diagnosis. Surgical revision was decided for all patients (osteotomy site curettage, bone graft and a more stable osteosynthesis using 2.0 screws). Bone healing was achieved in every case after revision surgery., Discussion: The incidence of non-union in our series was superior to that of published data (0.33 to 0.8%). Non-union always appeared in instable occlusion settings, and in three cases because of postoperative infection, but the main risk factor seemed to be osteosynthesis instability. Maxillary mobility is the key sign to screen for. Osteosynthesis material rupture or loosening is present every time but not always visible on standard radiography. 3D CT scan always allows observing non-union. Revision surgery combines curettage, bone graft, and a stronger osteosynthesis., (Copyright © 2010. Published by Elsevier Masson SAS.)

In honour of Professor Rossi's 80th birthday we review the development of our understanding of the immune and auto-immune nature of the pathogenesis of immune thrombocytopenic purpura (ITP). The immune aspects have been documented by postviral alterations of the cellular and humoral immune system, by new methods of specific auto-antibody detection against platelet glycoproteins and by the therapeutic effect of administering immunoglobulin concentrate from healthy blood donors. The various possible mechanisms of action of immunoglobulin treatment have led to use of this treatment as an alternative for other immune-related disorders. The treatment of severe chronic ITP in children, however, remains unsatisfactory. With a new international clinical and laboratory study of children and adolescents with early chronic ITP we are continuing the investigation of the pathogenesis and treatment of ITP.

Ambulatory blood pressure has been shown to be more reproducible than office blood pressure and thus to be more suited for studying the efficacy of antihypertensive drugs. In 34 untreated essential hypertensive subjects, we measured office and 24-h non-invasive or intra-arterial blood pressure twice over a 4-week interval; 24-h intra-arterial blood pressure was obtained by the Oxford method whereas 24-h non-invasive blood pressure was obtained by the automatic SpaceLabs 5300 device, with a 10 min (daytime) or 20 min (night-time) interval between measurements. The standard deviation of the mean difference (s.d.d.) between blood pressures obtained in each recording was taken as the reciprocal of blood pressure reproducibility. The s.d.d. was highest for office blood pressure and for single blood pressure readings taken from 24-h non-invasive recordings. The s.d.d. fell when the two 24-h average non-invasive blood pressures were considered. The fall was progressively greater as the number of ambulatory readings on which the average was calculated increased from two to 24, no further fall being observed when more than 24 values were considered. The maximal reduction in s.d.d. was 59% (systolic) and 42% (diastolic) as compared with the office s.d.d. The two 24-h mean values obtained by the intra-arterial approach were slightly more closely correlated than those obtained non-invasively. However, at comparable sampling rates, the s.d.d. was not substantially lower with 24-h intra-arterial blood pressure and including in the calculation the average of the thousand readings provided by this approach did not cause any further improvement.(ABSTRACT TRUNCATED AT 250 WORDS)

Spontaneous rupture of the vaginal vault is a rare event. Patients at risk are postmenopausal. Very often they show symptoms of an enterocele or have been operated before vaginally. Treatment is always by surgery. Whether it can be done vaginally or by laparotomy, depends on the local situation. To prevent recurrence, a double closure of the lesion is necessary, often combined with colpocleisis, colpectomy, sacropexie or obliteration of the "cul-de-sac".

In a prospective multicenter study 42 thrombocytopenic (less than 30 X 10(9) platelets/l) children with chronic idiopathic thrombocytopenic purpura (ITP) or with acute ITP, dependent on or refractory to corticosteroids, were given 0.4 g i.v. IgG/kg body weight/day on 5 consecutive days and thereafter once a week if the platelet count fell to less than 20 X 10(9)/l or if the patient bled. After the initial 5 days of i.v. IgG the platelets rose within a mean of 7-8 days to greater than 30 X 10(9)/l in all and to greater than 150 X 10(9)/l in 33 of 42 patients (79%). After a mean observation time of 26.6 months 26 of 42 patients (62%) showed a satisfactory long-term effect, i.e. no need for treatment for at least 6 months without bleeding and with no platelet counts below 20 X 10(9)/l. No difference in response rate was found between children with chronic and those with previously treated acute ITP. These results indicate that i.v. IgG could be used to control emergency situations, e.g. to stop bleeding or to prepare a patient for surgery. I.v. IgG also represents a good alternative to treatment modalities, such as splenectomy and/or the administration of cytostatic immunosuppressants with potentially serious side effects. In addition to the expected transient rise in serum IgG levels, i.v. IgG induced a more prolonged elevation of serum IgM. Platelet associated IgG, elevated before therapy, was correlated with the clinical long-term outcome.