The spectrum of inborn errors of immunity: a single tertiary center retrospective study in Alborz, Iran.

Summary: Background. Inborn errors of immunity (IEIs) are a group of heterogeneous disorders with inherited faults in the immune system that increase susceptibility to infections, malignancies, lymphoproliferation, and autoimmune/autoinflammatory disorders. Methods. We retrospectively studied the demographic characteristics, clinical features, and immunological profiles of the 90 IEIs patients, who were diagnosed and classified according to the European Society for Immunodeficiencies (ESID) and International Union of Immunological Societies (IUIS) criteria from July 2010 to June 2021. The study was carried out in the Non-communicable Diseases Research Center, Imam Ali Hospital, Alborz, Iran. Results. Within a period of 11 years, 53 (58.9%) males and 37 (41.1%) females were diagnosed and followed up for 20 IEI disorders. The median (IQR) age of onset, age of clinical diagnosis and diagnostic delay was 0.7 (0.08-2.0), 3.18 (1.0-8.0) and 1.5 (0.17-5.0) years, respectively. Twelve patients (36.4%) had a positive family history of IEI, and the majority of patients (84.5%) had recurrent infections. Pneumonia (51.7%) was the most common clinical manifestation among IEI patients, followed by skin complications (46.2%). The most frequently diagnosed IEI was immunoglobulin A deficiency (IgAD) (14.4%) and severe combined immunodeficiency (SCID) (11.1%). Predominantly antibody deficiencies group (36.7%) was the most common category, followed by combined immunodeficiencies with associated or syndromic features group (27.8%). Conclusions. IEIs have different patterns within populations with high consanguinity. There is a need to search for underlying genetic and epigenetic factors in most common IEIs in Alborz.

Invited review: Muscle protein breakdown and its assessment in periparturient dairy cows.

Mobilization of body reserves including fat, protein, and glycogen is necessary to overcome phases of negative nutrient balance typical for high-yielding dairy cows during the periparturient period. Skeletal muscle, the largest internal organ in mammals, plays a crucial role in maintaining metabolic homeostasis. However, unlike in liver and adipose tissue, the metabolic and regulatory role of skeletal muscle in the adaptation of dairy cows to the physiological needs of pregnancy and lactation has not been studied extensively. The functional integrity and quality of skeletal muscle are maintained through a constant turnover of protein, resulting from both protein breakdown and protein synthesis. Thus, muscle protein breakdown (MPB) and synthesis are intimately connected and tightly controlled to ensure proper protein homeostasis. Understanding the regulation of MPB, the catabolic component of muscle turnover, and its assessment are therefore important considerations to provide information about the timing and extent of tissue mobilization in periparturient dairy cows. Based on animal models and human studies, it is now evident that MPB occurs via the integration of 3 main systems: autophagy-lysosomal, calpain Ca2+-dependent cysteine proteases, and the ubiquitin-proteasome system. These 3 main systems are interconnected and do not work separately, and the regulation is complex. The ubiquitin-proteasomal system is the most well-known cellular proteolytic system and plays a fundamental role in muscle physiology. Complete degradation of a protein often requires a combination of the systems, depending on the physiological situation. Determination of MPB in dairy cows is technically challenging, resulting in a relative dearth of information. The methods for assessing MPB can be divided into either direct or indirect measurements, both having their strengths and limitations. Available information on the direct measures of MPB primarily comes from stable isotopic tracer methods and those of indirect measurements from assessing expression and activity measures of the components of the 3 MPB systems in muscle biopsy samples. Other indirect approaches (i.e., potential indicators of MPB), including ultrasound imaging and measuring metabolites from muscle degradation (i.e., 3-methylhistidine and creatinine), seem to be applicable methods and can provide useful information about the extent and timing of MPB. This review presents our current understanding, including methodological considerations, of the process of MPB in periparturient dairy cows.

Blood and adipose tissue steroid metabolomics and mRNA expression of steroidogenic enzymes in periparturient dairy cows differing in body condition.

In high-yielding dairy cows, the rapidly increasing milk production after parturition can result in a negative nutrient balance, since feed intake is insufficient to cover the needs for lactation. Mobilizing body reserves, mainly adipose tissue (AT), might affect steroid metabolism. We hypothesized, that cows differing in the extent of periparturient lipomobilization, will have divergent steroid profiles measured in serum and subcutaneous (sc)AT by a targeted metabolomics approach and steroidogenic enzyme profiles in scAT and liver. Fifteen weeks antepartum, 38 multiparous Holstein cows were allocated to a high (HBCS) or normal body condition (NBCS) group fed differently until week 7 antepartum to either increase (HBCS BCS: 3.8 ± 0.1 and BFT: 2.0 ± 0.1 cm; mean ± SEM) or maintain BCS (NBCS BCS: 3.0 ± 0.1 and BFT: 0.9 ± 0.1 cm). Blood samples, liver, and scAT biopsies were collected at week -7, 1, 3, and 12 relative to parturition. Greater serum concentrations of progesterone, androsterone, and aldosterone in HBCS compared to NBCS cows after parturition, might be attributed to the increased mobilization of AT. Greater glucocorticoid concentrations in scAT after parturition in NBCS cows might either influence local lipogenesis by differentiation of preadipocytes into mature adipocytes and/or inflammatory response.

Expression of specific signaling components related to muscle protein turnover and of branched-chain amino acid catabolic enzymes in muscle and adipose tissue of preterm and term calves.

Postnatal metabolism depends on maturation of key metabolic pathways around birth. In this regard, endogenous glucose production is impaired in calves born preterm. Concerning protein metabolism, the rates of protein turnover are greater during the neonatal period than at any other period of postnatal life. The mammalian target of rapamycin (mTOR) and the ubiquitin-proteasome system (UPS) are considered as the major regulators of cellular protein turnover. The objectives of this study were to investigate (1) the changes in plasma AA profiles, (2) the mRNA abundance of mTOR signaling and UPS-related genes in skeletal muscle, and (3) the mRNA abundance of branched-chain AA (BCAA) catabolic enzymes in skeletal muscle and adipose tissue in neonatal calves with different degree of maturation during the transition to extrauterine life. Calves (n = 7/treatment) were born either preterm (PT; delivered by cesarean section 9 d before term) or at term (T; spontaneous vaginal delivery) and were left unfed for 1 d. Calves in treatment TC were also spontaneously born but were fed colostrum and transition milk for 4 d. Blood samples were collected from all calves at birth and at 24 h of life. Additional blood samples were taken 2 h after feeding (26 h of life) for PT and T calves, and on d 4 of life for TC, to determine plasma glucose, urea, and AA. Tissue samples from 3 muscles [M. longissimus dorsi (MLD), M. semitendinosus (MST), and M. masseter (MM)], and kidney fat were collected following euthanasia at 26 h after birth (PT, T) or on d 4 of life (TC) at 2 h after feeding. The concentrations of the majority of plasma AA (Ala, Gln, Asn, Cit, Lys, Orn, Thr, and Tyr), nonessential AA, and total AA were greater during the first 24 h and also before and 2 h after feeding in PT than in T. The ratio of plasma BCAA to the aromatic AA (Tyr and Phe) was greatest in TC, followed by T, and least in PT. The mRNA abundance of mTOR and ribosomal protein S6 kinase 1 (S6K1) in MLD and MM was greater in PT and T than in TC. The mRNA abundance of muscle-specific ligases FBXO32 (F-box only protein 32) in the 3 different skeletal muscles and TRIM63 (tripartite motif containing 63) in MLD was greater in PT and T than in TC; in MM, TRIM63 mRNA was greatest in PT. The mRNA for BCKDHA and BCKDHB (the α and ß polypeptide of branched-chain α-keto acid dehydrogenase) in kidney fat was elevated in PT and T compared with TC, suggesting a possible enhancement of BCAA oxidation as energy source to cover the energetic and nutritional postnatal demands in PT and T in a starved state. The increased abundances of mTOR-associated signaling factors and muscle-specific ligase mRNA indicate a greater rate of protein turnover in muscles of PT and T in a starved state. Elevated plasma concentrations of several AA may result from enhanced muscle proteolysis and impaired conversion to glucose in the liver of PT calves.

Proteasome activity and expression of mammalian target of rapamycin signaling factors in skeletal muscle of dairy cows supplemented with conjugated linoleic acids during early lactation.

The mammalian target of rapamycin (mTOR) is a major regulator of protein synthesis via its main downstream effectors, ribosomal protein S6 kinase (S6K1) and eukaryotic initiation factor 4E binding protein (4EBP1). The ubiquitin-proteasome system (UPS) is the main proteolytic pathway in muscle, and the muscle-specific ligases tripartite motif containing 63 (TRIM63; also called muscle-specific ring-finger protein 1, MuRF-1) and F-box only protein 32 (FBXO32; also called atrogin-1) are important components of the UPS. We investigated 20S proteasome activity and mRNA expression of key components of mTOR signaling and UPS in skeletal muscle of dairy cows during late gestation and early lactation and tested the effects of dietary supplementation (from d 1 in milk) with conjugated linoleic acids (sCLA; 100 g/d; n = 11) compared with control fat-supplemented cows (CTR; n = 10). Blood and muscle tissue (semitendinosus) samples were collected on d -21, 1, 21, and 70 relative to parturition. Dry matter intake increased with time of lactation in both groups. It was lower in sCLA than in CTR on d 21, which resulted in a reduced calculated metabolizable protein balance. Most serum and muscle concentrations of AA followed time-related changes but were unaffected by CLA supplementation. In both groups, serum and muscle 3-methylhistidine (3-MH) concentrations and the ratio of 3-MH:creatinine increased from d -21 to d 1, followed by a decline on d 21. The mRNA abundance of MTOR on d 21 and 70 was greater in sCLA than in CTR. The abundance of 4EBP1 mRNA did not differ between groups but was upregulated in both on d 1. The mRNA abundance of S6K1 on d 70 was greater in CTR than in sCLA, but remained unchanged over time in both groups. The mRNA abundance of FBXO32 (encoding atrogin-1) on d 21 was greater in sCLA than in CTR. The mRNA abundance of TRIM63 (also known as MuRF1) showed a similar pattern as FBXO32 in both groups: an increase from d -21 to d 1, followed by a decline. The mRNA for the α (BCKDHA) and ß (BCKDHB) polypeptide of branched-chain α-keto acid dehydrogenase was elevated in sCLA and CTR cows on d 21, respectively, suggesting a role of CLA in determining the metabolic fate of branched-chain AA. For the mTOR protein, no group differences were observed. The abundance of S6K1 protein was greater across all time points in sCLA versus CTR. The antepartum 20S proteasome activity in muscle was elevated in both groups compared with postpartum, probably reflecting the start of protein mobilization before parturition. Plasma insulin concentrations decreased in both groups postpartum but to a greater extent in CTR than in sCLA, resulting in greater insulin concentrations in sCLA than in CTR. Thus, the greater abundance of MTOR mRNA and S6K1 protein in sCLA compared with CTR might be mediated by the greater plasma insulin postpartum. The upregulation of MTOR mRNA in sCLA cows on d 21, despite greater FBXO32 mRNA abundance, may reflect a simultaneous activation of both anabolic and catabolic signaling pathways, likely resulting in greater protein turnover.

Changes in tissue abundance and activity of enzymes related to branched-chain amino acid catabolism in dairy cows during early lactation.

Branched-chain α-keto acid dehydrogenase (BCKDH) complex catalyzes the irreversible oxidative decarboxylation of branched-chain α-keto acids. This reaction is considered as the rate-limiting step in the overall branched-chain amino acid (BCAA) catabolic pathway in mammals. For characterizing the potential enzymatic involvement of liver, skeletal muscle, adipose tissue (AT), and mammary gland (MG) in BCAA metabolism during early lactation, tissue and blood samples were examined on d 1, 42, and 105 after parturition from 25 primiparous Holstein cows. Serum BCAA profiles were analyzed and the mRNA and protein abundance as well as the activity in the different tissues were assessed for the BCAA catabolic enzymes, partly for the branched-chain aminotransferase and completely for BCKDH. Total BCAA concentration in serum was lowest on d 1 after parturition and increased thereafter to a steady level for the duration of the experiment. Pronounced differences between the tissues were observed at all molecular levels. The mRNA abundance of the mitochondrial isoform of branched-chain aminotransferase (BCATm) was greatest in AT as compared with the other tissues studied, indicating that AT might be an important contributor in the initiation of BCAA catabolism in dairy cows. From the different subunits of the BCKDH E1 component, only the mRNA for the ß polypeptide (BCKDHB), not for the α polypeptide (BCKDHA), was elevated in liver. The BCKDHA mRNA abundance was similar across all tissues except muscle, which tended to lower values. Highest BCKDHA protein abundance was observed in both liver and MG, whereas BCKDHB protein was detectable in these tissues but could not be quantified. Adipose tissue and muscle only displayed abundance of the α subunit, with muscle having the lowest BCKDHA protein of all tissues. We found similarities in protein abundance for both BCKDH E1 subunits in liver and MG; however, the corresponding overall BCKDH enzyme activity was 7-fold greater in liver compared with MG, allowing for hepatic oxidation of BCAA transamination products. Reduced BCKDH activity in MG associated with no measurable activity in AT and muscle may favor sparing of BCAA for the synthesis of the different milk components, including nonessential AA. Deviating from previously published data on BCAA net fluxes and isotopic tracer studies in ruminants, our observed results might in part be due to complex counter-regulatory mechanisms during early lactation.

Effects of flushing and hormonal treatment on reproductive performance of Iranian Markhoz goats.

Forty-eight Iranian Markhoz goats were allocated to six groups (n = 8) to study the effect of flushing and hormonal treatments on reproductive performance. Treatments were divided into two categories including, hormonal treatments and flushing. The goats in each group were fed the same basal ration and received one of the following treatments: Groups A and B--injection of GnRH and equine chorionic gonadotropin (eCG) respectively; Groups C, D and E--a supplement of barley grain, soybean oil and sunflower oil in flushing diets, respectively, were offered and Group F--control (only received basal diet). In the flushing treatments, only the source of energy was different between rations. Both hormonal treatments and flushing treatments improved fertility and kidding rates. Treatment B with 16 and control with seven kids represented the highest and the lowest number of progeny respectively. Among flushing treatments, group C resulted in the highest number of kids being 15. Oestrogen levels in follicular phase increased with the injection of eCG and consumption of barley grain. GnRH injection and consumption of oil sources in the diet increased blood progesterone levels during ovulation and post-ovulation periods. Under current market conditions, using hormone or flushing can be profitable for Markhoz goats farmers.

Plasma leptin and mRNA expression of lipogenesis and lipolysis-related factors in bovine adipose tissue around parturition.

The objective was to study changes in plasma leptin concentration parallel to changes in the gene expression of lipogenic- and lipolytic-related genes in adipose tissue of dairy cows around parturition. Subcutaneous fat biopsies were taken from 27 dairy cows in week 8 antepartum (a.p.), on day 1 postpartum (p.p.) and in week 5 p.p. Blood samples were assayed for concentrations of leptin and non-esterified fatty acids (NEFA). Subcutaneous adipose tissue was analysed for mRNA abundance by real-time qRT-PCR encoding for leptin, adiponectin receptor 1 (AdipoR1), adiponectin receptor 2 (AdipoR2), hormones-sensitive lipase (HSL), perilipin (PLIN), lipoprotein lipase (LPL), acyl-CoA synthase long-chain family member 1 (ACSL1), acetyl-CoA carboxylase (ACC), fatty acid synthase (FASN) and glycerol-3-phosphate dehydrogenase 2 (GPD2). Body weight and body condition score of the cows were lower after parturition than before parturition. The calculated energy balance was negative in week 1 and 5 p.p., with higher negative energy balance in week 1 p.p. compared with that in week 5 p.p. On day 1 p.p., highest concentrations of NEFA (353.3 µmol/l) were detected compared with the other biopsy time-points (210.6 and 107.7 µmol/l, in week 8 a.p., and week 5 p.p. respectively). Reduced plasma concentrations of leptin during p.p. when compared with a.p. would favour increasing metabolic efficiency and energy conservation for mammary function and reconstitution of body reserves. Lower mRNA abundance of ACC and FASN expression on day 1 p.p. compared with other biopsy time-points suggests an attenuation of fatty acid synthesis in subcutaneous adipose tissue shortly after parturition. Gene expression of AdipoR1, AdipoR2, HSL, PLIN, LPL, ACSL1 and GPD2 was unchanged over time.

Determining the location of hip joint centre: application of a conchoid's shape to the acetabular cartilage surface of magnetic resonance images.

Preoperative planning, or intraoperative navigation of hip surgery, including joint-preserving procedures such as osteotomy or joint-replacing procedures such as total arthroplasty, needs to be performed with a high degree of accuracy to ensure a successful outcome. The ability to precisely localise the hip joint rotation centre may prove to be very useful in this context. The human hip joint has been shown to be a conchoid shape, and therefore the accurate location of the hip joint centre (HJC) cannot be computed simply as the centre of a sphere. This study describes a method for determining the HJC by applying a conchoid shape to the acetabular cartilage surface of magnetic resonance images, in order to increase the accuracy of the HJC location which had previously been calculated by a functional method using reconstructed three-dimensional surface bony models. By approximating a conchoid shape to the acetabulum, it was possible to compensate for HJC calculation errors.

Assessment of arthroscopic management of femoroacetabular impingement. A prospective multicenter study.

INTRODUCTION: Surgical treatment of femoroacetabular impingement can be performed under arthroscopic control, to limit associated morbidity. Encouraged by recent good reports, arthroscopy is replacing alternative techniques for this indication. HYPOTHESIS: Arthroscopy enables femoroacetabular impingement to be corrected with a low rate of associated morbidity. AIM OF STUDY: To assess the indications for and quality of the technique and its impact on preliminary results and complications. To investigate preoperative prognostic factors. PATIENT AND METHODS: One hundred and eleven hips in 110 patients (78 male, 32 female; mean age, 31 years) were operated on under arthroscopic control for femoroacetabular impingement, by six senior surgeons. Sixty-five patients showed no radiographic sign of osteoarthritis, and 36 showed grade-1 early osteoarthritis on the Tönnis scale. RESULTS: Mean WOMAC score rose from 60.3 preoperatively to 83 (p<0.001) at a mean 10 months' FU (range, 6-18 mo). Seventy-seven percent of patients were satisfied or very satisfied with their result. Patients with early osteoarthritis had significantly lower WOMAC and satisfaction scores than those free of osteoarthritis. Operative crossover to open surgery occurred in only one case. Five patients (4%) had revision: total hip replacement or resurfacing. There were seven complications (6%): three cases of heterotopic ossification, one of crural palsy, one of pudendal palsy, one of labium majus necrosis, and one non-displacement stress fracture of the femoral head/neck junction (managed by non-weight-bearing). There was no palsy of the territory of the lateral cutaneous nerve of the thigh. DISCUSSION: Results confirmed the efficacy and low associated morbidity of arthroscopy in the management of femoroacetabular impingement. Short-term functional results matched those of the literature. Planning and assessment seem not yet to be fully standardized. Preoperative osteoarthritis on X-ray was associated with poorer functional results. This attitude does not seem to be indicated for hips showing evolved osteoarthritis (>grade 1).

Hip arthroscopy technique and complications.

Hip arthroscopy is not a new technique but the seemingly limited indications and technical challenges involved have discouraged many since Burman documented his initial experiences (Burman, 1991). The current renaissance is largely driven by the expanding indications particularly in the management of femoroacetabular impingement. The figures from our own unit illustrate this well with 40% of hip arthroscopies in 1990 being purely diagnostic as compared with less than 5% at the present time. The focus of this chapter is the technical aspects of hip arthroscopy and the potential complications that can occur.

Gene expression of tumour necrosis factor and insulin signalling-related factors in subcutaneous adipose tissue during the dry period and in early lactation in dairy cows.

Gene expression of adipose factors, which may be part of the mechanisms that underlie insulin sensitivity, were studied in dairy cows around parturition. Subcutaneous fat biopsies and blood samples were taken from 27 dairy cows in week 8 antepartum (a.p.), on day 1 postpartum (p.p.) and in week 5 p.p. In the adipose tissue samples, mRNA was quantified by real-time reverse transcription polymerase chain reaction for tumour necrosis factor alpha (TNFα), insulin-independent glucose transporter (GLUT1), insulin-responsive glucose transporter (GLUT4), insulin receptor, insulin receptor substrate 1 (IRS1), insulin receptor substrate 2 (IRS2), regulatory subunit of phosphatidylinositol-3 kinase (p85) and catalytic subunit of phosphatidylinositol-3 kinase. Blood plasma was assayed for concentrations of glucose, ß-hydroxybutyric acid, non-esterified fatty acids (NEFA) and insulin. Plasma parameters followed a pattern typically observed in dairy cows. Gene expression changes were observed, but there were no changes in TNFα concentrations, which may indicate its local involvement in catabolic adaptation of adipose tissue. Changes in GLUT4 and GLUT1 mRNA abundance may reflect their involvement in reduced insulin sensitivity and in sparing glucose for milk synthesis in early lactation. Unchanged gene expression of IRS1, IRS2 and p85 over time may imply a lack of their involvement in terms of insulin sensitivity dynamics. Alternatively, it may indicate that post-transcriptional modifications of these factors came into play and may have concealed an involvement.

Usefulness of the head-up tilt test in distinguishing neurally mediated syncope and epilepsy in children aged 5-20 years old.

Many nonepileptic disorders may mimic epilepsy by history or clinical presentation. Neurally mediated syncope is one of the most important conditions that might be difficult to differentiate from epilepsy on clinical grounds. We investigated the value of the head-up tilt test (HUT) to diagnose syncope in epileptic children. We studied 40 patients (18 girls and 22 boys) between 5 and 20 years old (mean, 11.5 +/- 3.5) who had a previous diagnosis of epilepsy. All patients underwent a HUT test. The HUT test was positive in 26 patients (65%). No statistical difference was observed between the tilt positive and negative groups in sex, age, provocating factors, associated symptoms, family history of syncope and heart disease, findings in physical examination, and electroencephalogram result. There was a history in favor of true syncope in 58% of tilt positive patients compared to 14% of tilt negative patients (p < 0.05). Also, family history of seizure was more frequent in tilt positive patients (p < 0.05). After 18 +/- 6 months of follow-up, 18 of 26 patients with a positive tilt test were completely asymptomatic. Inadequate history taking and overemphasis on positive family history for seizures were important causes of misdiagnosis of epilepsy in our study. The HUT test is a simple, noninvasive diagnostic tool for distinguishing syncope and epilepsy in children and should be considered early in the diagnostic plan and for determining management of selected patients with a history of drop attack and loss of consciousness.

[Hip dysplasia and misalignment in patients aged 20-50 years]. / Dysplasie et conflits de hanche de 20 à 50 ans.

Anterior hip pain in young adult (20 to 50) has two main causes: secondary osteo-arthritis on development dysplasia of the hip, and femoro-acetabular impigement (FAI). This symposium had two parts: the first one analyses long-term results of non-prosthetic surgery (283 osteotomies and shelfs at 15 years FU). The second part concerned the different syndromes with acute anterior hip pain, especially due to FAI and to labral tears.In hip dysplasia, 56 shelf operations, 100 proximal femoral varus osteotomies associated or not with a shelf arthroplasty,and 127 Chiari osteotomies were examined with 10 years minimum follow-up. Only 15% of patients were lost at follow-up before 10 years and average follow-up was 15 years. Results were considered as satisfactory when the Merle d'Aubigne rating was 15/18 or more. The 3 main factors of good prognosis were: a complete correction of both femoral and acetabular dysplasia; age at operation under 40; a moderate arthritis (grade I or II according to De Mourgues and Patte). In single acetabular dysplasia with 3 favorable prognosis factors(no coxa valga, age under 40, arthritis 1 or 2), 85% good results were achieved at 15 years. When patients were over 40 at operation, or in arthritis grade over 2, only 55% of good results were observed. Varus osteotomies, associated or not with shelf arthroplasties, achieved also 85% goods results at 15 years when the 3 favorable prognosis factors were present. Similar good results were also obtained by Chiari osteotomy, but this operation was associated with 12% complications, and more that 25% of lasting limping. Therefore, with 85% good results at 15 years (and often over 20 years), non prosthetic surgery performed at 30-35 years, achieved better functional results than total hip arthroplasty, longer lasting, and not jeopardizing any further possibility of prosthetic surgery.As concerns acute anterior pain of the hip, the clinical and imaging patterns of the different syndromes have been precised: femoro-acetabular impigement by cam (or by pincer), labral tears in hip dysplasia. There were distinguished from the other secondary impigements, for example by acetabular malposition due to pelvis anteflexion or by other hip diseases: overuse arthritis, coxa retrorsa, etc. Several examples of typical syndromes were presented to support the recommended imaging techniques. The results of the speakers with different surgical treatments were reported as well as concerns open surgery than arthroscopic treatment (60 cases).

[Hooked roof cup in revision of difficult loose hip prosthesis cups. Results after a minimum of 10 years]. / Die Hakendachschale in der Revision schwieriger gelockerter Hüftprothesenpfannen. Resultate nach mindestens 10 Jahren.

The long-term results of acetabular revision after total hip arthroplasty (THA) with the use of a reinforcement ring with hook were evaluated. The study included 57 cases of surgery dating back 10 years or more. Of a total of 54 patients (57 operated hips), 18 patients (19 hips) died during the 10-year period and 2 patients (2 hips) were completely lost to follow-up. At the time of the revision surgery, the mean age of the remaining 34 patients (36 hips) was 62.5 years (range: 47-80). A THA revision was done in 25 cases and an acetabular revision only in 11 cases. The most common acetabular defect was a combined segmental and cavitary defect (n = 19), and in three cases there was pelvic discontinuity. Autologous or homologous cancellous bone grafts were used to fill acetabular cavities in 17 hip joints. Structured bone grafts, predominantly homologous bone, were used in ten cases for acetabular reconstruction. At a mean follow-up of 11.4 years (range: 10-14.5) three hip joints (8%) had undergone further revision. The revisions were done for aseptic loosening of the acetabular component in two cases and a septic loosening of both components in one case. Three further cases (8%) revealed signs of acetabular loosening. Two of these three patients were symptomatic but refused further revision surgery. In the 33 unrevised hip joints, a good or excellent clinical result with a d'Aubingé score of more than 14 points was found in 30 cases (92%). Osseous acetabular reconstruction with the use of a reinforcement ring leads to favorable results compared to other techniques. In the authors' opinion, this technique is preferable to those using oversized cups without osseous reconstruction of the acetabulum.