ADAMTS13 recovery in acute thrombotic thrombocytopenic purpura after caplacizumab therapy.

ABSTRACT: Caplacizumab prevents the interaction between von Willebrand factor and platelets and is used to treat immune thrombotic thrombocytopenic purpura (iTTP). Its administration has been associated with a delay in ADAMTS13 activity restoration after plasma exchange (PEX) suspension. We analyzed the outcomes of 113 iTTP episodes, 75 of which were treated with caplacizumab, in 108 patients from the Spanish Registry of Thrombotic Thrombocytopenic Purpura. Caplacizumab shortened the time to platelet count normalization and reduced PEX requirement, exacerbations, and relapses. There was no difference in the time to achieve ADAMTS13 activity ≥20% after PEX end between caplacizumab-treated and nontreated episodes (median [interquartile range], 14.5 [7.7-27.2] vs 13.0 [8.0-29.0] days, P = .653). However, considering the 36 episodes in which caplacizumab was started ≤3 days after iTTP diagnosis, the time for ADAMTS13 restoration from PEX end was higher than in those episodes in which caplacizumab was started >3 days after iTTP diagnosis (20.0 [12.0-43.0] vs 11.0 [3.5-20.0] days, P = .003) or than in non-caplacizumab-treated episodes (P = .033). This finding could be related to a significantly shorter duration of PEX in early caplacizumab-treated episodes than in late caplacizumab-treated episodes (5.5 [4.0-9.0] vs 15.0 [11.0-21.5] days, P < .001) or non-caplacizumab-treated episodes (11.0 [6.0-26.0] days, P < .001). There were no differences in time to ADAMTS-13 restoration from PEX start (28.0 [17.2-47.5], 27.0 [19.0-37.5] and 29.5 [15.2-45.0] days in early caplacizumab-treated, late caplacizumab-treated and non-caplacizumab-treated episodes). Early administered caplacizumab does not prevent the requirement for immunosuppression but has beneficial effects by shortening PEX requirement without major safety concerns.

Largest comparison between onset and relapses of acquired thrombotic thrombocytopenic purpura reveals severe neurological involvement and worse analytic parameters at debut.

It has been proposed that the onset of Acquired Thrombotic Thrombocytopenic Purpura (iTTP) is more severe than subsequent relapses; however, existing studies have limitations. We conducted a retrospective observational study to compare analytical and clinical severity of onset and relapse aTTP cases between 2012 and 2023. A total of 370 episodes of aTTP were analyzed, comprising 272 at initial diagnosis and 98 relapses. At onset, analytical parameters indicative of severity (low hemoglobin, low platelet count, and increased LDH) were significantly worse; patients had severe neurological symptoms (p<0.001) and ≥ 3 points in the TMA mortality score (p<0.001). In conclusion, the onset of aTTP is associated with worse analytical parameters and severe neurological involvement.

Effects of a 10% dimethyl sulfoxide solution on radiocarpal joint amikacin pharmacokinetics during intravenous regional limb perfusion in standing sedated horses.

OBJECTIVE: To determine the effect of a 10% dimethyl sulfoxide (DMSO) solution on the peak concentration (CMAX ) of amikacin in the radiocarpal joint (RCJ) during intravenous regional limb perfusion (IVRLP) compared with 0.9% NaCl. STUDY DESIGN: Randomized crossover study. ANIMALS: Seven healthy adult horses. METHODS: The horses underwent IVRLP with 2 g of amikacin sulfate diluted to 60 mL using a 10% DMSO or 0.9% NaCl solution. Synovial fluid was collected from the RCJ at 5, 10, 15, 20, 25, and 30 minutes after IVRLP. The wide rubber tourniquet placed on the antebrachium was removed after the 30 min sample. Amikacin concentrations were quantified by a fluorescence polarization immunoassay. The mean CMAX and time to peak concentration (TMAX ) of amikacin within the RCJ were determined. A one-sided paired t-test was used to determine the differences between treatments. The significance level was p < .05. RESULTS: The mean ± SD CMAX in the DMSO group was 1361.8 ± 593 µg/mL and in the 0.9% NaCl group it was 860 ± 481.6 µg/mL (p = .058). Mean TMAX using the 10% DMSO solution was 23 and 18 min using the 0.9% NaCl perfusate (p = .161). No adverse effects were associated with use of the 10% DMSO solution. CONCLUSION: Although there were higher mean peak synovial concentrations using the 10% DMSO solution no difference in synovial amikacin CMAX between perfusate type was detected (p = .058). CLINICAL SIGNIFICANCE: Use of a 10% DMSO solution in conjunction with amikacin during IVRLP is a feasible technique and does not negatively affect the synovial amikacin levels achieved. Further research is warranted to determine other effects of using DMSO during IVRLP.

Does size matter? Two new deletions in the HBB gene cause ß0-thalassemia.

Most ß-thalassemias are caused by mutations involving one or a limited number of nucleotides within the gene or its adjacent regions. They can be substitutions or deletions; in these cases, the loss ranges from a single nucleotide to even the entire HBB gene, so we wonder if the phenotype is due to the size of the deletion or the location of the mutation. To clarify this, we present two new deletions in the ß-globin gene that cause ß0-thalassemia. The hematological parameters were determined with an automated cell counter; the Hb A2 and Hb F levels were measured by performance liquid chromatography. Hemoglobins were analyzed by capillary zone electrophoresis (Sebia Capillarys Flex system) and ion-exchange HPLC (BioRad Variant II ß-thalassemia Short Program). Molecular characterization was performed by automatic Sanger sequencing. The screening of common α-thalassemia point mutations and deletions in the world (21 in total) were carried out using multiplex PCR followed by reverse-hybridization with a commercial Alpha-Globin StripAssay kit. We have characterized two new mutations-(1) 1-bp deletion [CD61/62(-G)] [HBB:c.186_187delG], (2) 105-bp deletion [IVS-2-nt767-CD111] [HBB:c.316-84_333del]-and we have described, for first time in Spain, the 25-bp deletion [ß nts 252 - 276 deleted] [HBB:c.93-22_95del] mutation. These mutations were classified as pathogenic by UniProt Variants confirmed according to the American College of Medical Genetics and Genomics guidelines. These mutations present a phenotype compatible with ß0-thalassemia, supported by hematological parameters that correlate the degree of reduction in the synthesis of the ß-globin chain. Identification of this type of mutation is important for genetic counselling of partners where both are carriers, so that they are aware of the genetic risk of having affected children, allowing them to take an informed decision about their reproductive choices.

Hb Nivaria: A New Hemoglobin Variant with a Shortened α-Globin Chain [α139(HC1)Lys→Stop; HBA1: c.418A>T].

We report a novel hemoglobin (Hb) variant found in a Spanish individual from Santa Cruz de Tenerife, the Canary Islands in Spain. The proband was a 39-year-old male. High performance liquid chromatography (HPLC) displayed an unknown peak (19.3%) at a retention time of 1.3 min. eluting before Hb A0. Capillary zone electrophoresis (CZE) showed an abnormal peak (20.0%) in zone 12. Direct DNA sequencing of the α-globin genes revealed heterozygosity for a nonsense mutation at codon 139 (AAA>TAA), causing a lysine to stop codon substitution [α139(HC1)Lys→Stop; HBA1: c.418A>T]. We decided to name the variant Hb Nivaria (Tenerife) for the place of birth and residence of the proband.

In vitro efficacy of a 0.2% polyhexamethylene biguanide-impregnated gauze dressing against pathogenic bacterial isolates found in horses.

OBJECTIVE: To determine the ability of 0.2% polyhexamethylene biguanide (PHMB)-impregnated gauze to inhibit the growth of bacteria isolated from equine infected sites. STUDY DESIGN: In vitro study. METHODS: Nine bacterial isolates were obtained from cultures submitted from equine patients presenting with penetrating injuries of the hoof (n = 4), septic osteitis (n = 1), synovial sepsis (n = 1), wounds (n = 2), and incisional infection following laparotomy (n = 1). Two standardized strains were also included. A standard inoculum of each isolate was placed on 12 Muller-Hinton agar plates. Squares (2.5 cm × 2.5 cm) of 0.2% PHMB-impregnated (n = 6) and nonimpregnated control gauze (n = 6) were placed on inoculated agar plates. Bacterial growth under each gauze square was assessed after a 24-h incubation period and areas of inhibition were measured to a standardized scale, using image-processing software. Mean ± SD growth inhibition (%) using 0.2% PHMB-impregnated gauze was compared to the nonimpregnated gauze for each isolate using Student's t test (p < .05). RESULTS: The 0.2% PMHB-impregnated gauze inhibited the growth of Staphylococcus spp. (n = 4) by 33%-83.1% and that of Escherichia coli spp. (n = 4) by 6.5%-37%. There was no inhibition of growth of Pseudomonas aeruginosa or either Enterococcus spp. CONCLUSION: The 0.2% PHMB-impregnated dressing tested here inhibited the growth of staphylococcal and E. coli isolates, but the magnitude of inhibition varied between strains. CLINICAL RELEVANCE: These results justify in vivo studies to evaluate the ability of the dressing to reduce the bacterial growth of common equine bacterial pathogens in clinical practice.

Orthopedic Infections-Clinical Applications of Intravenous Regional Limb Perfusion in the Field.

For the equine veterinarian, orthopedic emergencies are a common occurrence in clinical practice, with traumatic wounds of the distal limb and penetrating injuries of the hoof being some of the most common medical conditions to affect horses. Intravenous regional limb perfusion is a technique widely used for the treatment of orthopedic infections in horses. The objectives of this review are to discuss some of the clinical applications for this treatment modality in the field and to review the technique for the practitioner.

Nondeletional α-Thalassemia: Two New Mutations on the α2 Gene.

About 10.0% of α-thalassemia (α-thal) cases are due to point mutations, small deletions, or insertions of one or more bases on the α genes that can alter mRNA processing at the transcription, translation, or post-translation level; these cases are called nondeletional α-thalassemias (α-thal). Most occur within the domain of the α2 gene without changes in the expression of the α1 gene. We present two new frameshift mutations on the HBA2 gene, associated with a nondeletional α-thal phenotype. The probands were referred to our clinic because of persistent microcytosis and hypochromia. The molecular characterization was performed by automatic sequencing of the α-globin genes. Two new mutations were detected on the HBA2 gene; HBA2: c.85delG, p.(Ala29fs*21), and HBA2: c.268_280delCACAAGCTTCGGG, p.(His90Trpfs*9). These new mutations cause a change of the reading frame, the first on codon 28 and the second from codons 89 to 93. In the first mutation, the result is an altered amino acid sequence and a premature termination codon at position 87, while the elimination of 13 bp generates a protein of 95 residues and in this case, the premature termination codon is at position 96. These types of mutation are among the most damaging changes to the coding of a protein. Not only do they lead to changes in the length of the polypeptide, but they also vary the chemical composition, which would result in a nonfunctional protein. The importance of identifying these new mutations lies in their possible association with α0-thal, which could lead to a severe thalassemia.

Incidence of incisional complications after exploratory celiotomy in equids affected with enterolithiasis.

This study reports the prevalence of and risk factors for incisional complications in equids after ventral midline celiotomy for enterolithiasis. This study covered the years 2008 to 2015 and included 72 equids. Enteroliths were removed from the ascending or descending colon through 1 or more enterotomies. Complications were defined as surgical site infection and/or incisional hernia formation. Follow-up by telephone questionnaire or medical records determined that 10/72 (13.9%) equids experienced complications, with 6/72 (8.3%) developing a surgical site infection and 5/72 (6.9%) a hernia. Seven of ten were presented for chronic abdominal discomfort (> 24 hours), and 8/10 had right dorsal colon and pelvic flexure enterotomies. All equids that developed an incisional hernia and 4 with surgical site infection had enteroliths > 15 cm diameter removed from the right dorsal colon. Antimicrobial powder applied to the ventral midline incision during closure significantly reduced incisional complications. Removal of > 15 cm diameter enteroliths from the right dorsal colon may predispose to postoperative incisional complications.

Incidence des complications incisionnelles après une céliotomie exploratoire chez des équidés atteints d'entérolithiase. Cette étude rapporte la prévalence et les facteurs de risque des complications incisionnelles chez les équidés opérés pour l'entérolithiase en utilisant une celiotomie médiane ventrale de 2008 à 2015. Soixantedouze équidés ont été inclus. Les entérolithes ont été retirés du côlon ascendant ou descendant par ≥ 1 entérotomies. Les complications étaient définies comme une infection du site opératoire et/ou la formation d'une hernie incisionnelle. Le suivi a été obtenu par questionnaire téléphonique ou par dossiers médicaux. Dix des 72 (13,9 %) des équidés ont eu des complications, dont 6/72 (8,3 %) ont développé une infection du site opératoire et 5/72 (6,9 %) une hernie. Sept sur 10 ont été présentés pour un malaise abdominal chronique (> 24 heures) et 8/10 avaient des entérotomies du côlon dorsal droit et de la flexion pelvienne. Tous les équidés ayant développé une hernie incisionnelle et quatre avec une infection du site opératoire avaient des entérolithes > 15 cm de diamètre prélevés du côlon dorsal droit. La poudre antimicrobienne appliquée sur l'incision médiane ventrale lors de la fermeture réduisait significativement les complications incisionnelles. Le retrait d'entolithes de > 15 cm de diamètre du côlon dorsal droit peut prédisposer aux complications incisionnelles postopératoires.(Traduit par les auteurs).

Characterization of deletional and non-deletional alpha globin variants in a large cohort from Spain between 2009 and 2014.

The hemoglobinopathies are a group of disorders passed down through families (inherited) in which there is abnormal production or structure of the hemoglobin molecule. They are among the most common inherited diseases around the world. Those that produce abnormal hemoglobin are called structural hemoglobinopathies while thalassemia is another type of disorder that is caused by a defect in the gene production of the globin chains. In a study ambispective comprising 1623 patients, 153 subjects showed an abnormal hemoglobin and 1470 with hypochromic and microcytic anemia, and of these 1470, 23 patients were studied for simultaneously α-thalassemias and structural hemoglobinopathies. Among the α-thalassaemia cases, 1282 cases (87.2%) were deletional α-thalassemia, 172 cases (11.7%) were non-deletional α-thalassemia, and 16 cases (1.1%) were deletional and non-deletional α-thalassamias simultaneously. Thus, approximately 12% of the cases were non-deletional α-thalassaemia. Clinical diagnosis, only 19 severe cases (1 hydrops fetalis and 18 instances of Hb H disease), 1200 thalassamias traits, and 160 thalassaemia silent carriers were recorded within the α-thalassaemia. Regarding structural hemoglobinopathies, there were only 2 cases of hemoglobinopathies with low oxygen affinity and 1 case of hemoglobin M; the remaining 150 were silent hemoglobinopathies. Non-deletional α-thalassaemia represented 12% of all α-thalassemias in our region; the most common deletion in our area was the 3.7-kb deletions, followed by Asian --(SEA) and --(FIL). The alterations responsible for non-deletional α-thalassaemia are most represented by the Hph and Hb Groene Hart and, in the case of structural hemoglobinopathies, Hb Le Lamentin and Hb J-Paris.

Predictive value of plasma and peritoneal creatine kinase in horses with strangulating intestinal lesions.

OBJECTIVE: To determine the ability of plasma and peritoneal creatine kinase (CK) to predict the presence of a strangulating lesion in horses presented for colic. STUDY DESIGN: Prospective clinical study. ANIMALS: Ten healthy control horses and 61 clinical colic cases. METHODS: Creatine kinase activity was measured in peritoneal fluid and plasma of 10 healthy horses and 61 horses presenting for colic (40 horses with nonstrangulating lesions and 21 horses with strangulating lesions). Information on other blood and peritoneal fluid variables, signalment, results from the physical examination, outcome, requirement for surgery, and lesion location and type were retrieved from the medical records of horses presenting for colic. RESULTS: A peritoneal CK cutoff level of 16 IU/L yielded a sensitivity of 95.2% and a specificity of 84.6% (positive predictive value [PPV] = 76.9% and negative predictive value [NPV] = 97%, respectively) for predicting a strangulating lesion. A peritoneal lactate cutoff level of 3.75 mmol/L yielded a sensitivity of 81% and a specificity of 92% (PPV = 85% and NPV = 90%, respectively) for predicting a strangulating lesion. CONCLUSION: Peritoneal CK concentration was a sensitive indicator of the presence of a strangulating lesion in horses presenting with colic, whereas peritoneal lactate concentration was a more specific indicator. CLINICAL SIGNIFICANCE: Measuring CK in peritoneal fluid may be a useful adjunct to clinical case presentation to accelerate the diagnosis and definitive treatment of horses presenting with strangulating intestinal lesions, thereby improving their outcome.

Preoperative factors associated with resection and anastomosis in horses presenting with strangulating lesions of the small intestine.

OBJECTIVE: To determine if preoperative and intraoperative clinical variables correlate with resection and anastomosis (RA) in horses presenting with strangulating small intestinal (SI) lesions. STUDY DESIGN: Retrospective case series. ANIMALS: Horses undergoing exploratory celiotomy for a strangulating SI lesion (n = 243). METHODS: The records of horses undergoing surgical correction for strangulating SI lesions from January 1, 2000 to December 31, 2017 were reviewed. Horses were categorized as treated with RA or not treated with RA. Obtained information included history, signalment, clinical findings, diagnostic test results, intraoperative findings, and survival to discharge. Univariable and multivariable analyses were used to compare data between horses treated with RA vs those not treated with RA (P = .05). RESULTS: In total, 172 SI lesions were resected, and 71 were not resected. Horses that had gastric reflux at admission to the hospital (odds ratio [OR] 5.56; 95% CI 1.76, 17.59) and horses with amotile SI according to abdominal ultrasonography (OR 9.69; 95% CI 3.09, 30.37) were more likely to undergo RA. Increased difference between peritoneal fluid and blood lactate was higher in horses that underwent RA (OR 1.55; 95% CI 1.12, 2.13). Survival to hospital discharge was lower in the group of horses that underwent RA compared with horses that did not undergo RA. CONCLUSION: Resection and anastomosis of strangulating SI lesions was associated with several preoperative variables. CLINICAL SIGNIFICANCE: This study highlights the value of preoperative diagnostic procedures in aiding intraoperative decision making and prognostication in horses presenting with strangulating SI lesions.

Development and validation of a multivariable prediction rule for detecting a severe acquired ADAMTS13 activity deficiency in patients with thrombotic microangiopathies.

BACKGROUND: Thrombotic microangiopathies (TMAs) are a group of diseases that have different aetiologies and treatments, but a clinical differential diagnosis remains difficult. Among TMAs, thrombotic thrombocytopenic purpura (TTP) is characterised by a severe ADAMTS13 functional deficiency. However, assays exploring ADAMTS13 activity are limited to some specialised laboratories. Our objective was to develop and validate a diagnostic method for TTP in adult patients with TMA. METHODS: We generated a multivariable model (four predictors) on a cohort of 174 TMA patients in order to predict an ADAMTS13 activity deficiency (AUC of 0.927). The multivariable model was simplified into a binary rule to facilitate the interpretation of the predictions. There were two scenarios for a patient: (1) Predicted ADAMTS13 deficiency; if the patient met four conditions simultaneously (platelets ≤44×109/L, creatinine ≤2 mg/dL (≤176.84 µmol/L) for males or ≤1.9 mg/dL (≤168 µmol/L) for females, age ≤68 years and no history of haematopoietic stem cell transplant [HSCT]); or (2) Predicted "normal" activity; if any of the above conditions are not met. This rule was validated on a second cohort of 86 patients and performed with sensitivity of 87.7% and specificity of 92.7%. RESULTS AND CONCLUSIONS: This could lead to the earlier confirmation or rapid exclusion of TTP when ADAMTS13 testing is not avalilable, facilitating a more suitable therapy based on the aetiology of the TMA.

Catastrophic gastric rupture in a horse secondary to psyllium pharmacobezoars.

A mare was euthanized because of gastric rupture secondary to complete duodenal obstruction by 2 bezoars located in the pylorus and proximal duodenum. Infrared spectroscopy showed that the bezoars were composed of psyllium. The mare had been receiving treatment with a pelleted psyllium product at 4 times the recommended dosage. Veterinarians should be aware that treatment of colic in horses with pelleted psyllium products could be associated with gastric impaction.

Rupture gastrique catastrophique secondaire à des pharmacobézoards de psyllium en boulettes chez un cheval. Une jument a été euthanasiée en raison d'une rupture gastrique secondaire à une obstruction duodénale complète par deux bézoards situés dans le pylore et le duodénum proximal. La spectroscopie infrarouge a montré que les bézoards se composaient de psyllium. La jument avait reçu un traitement composé d'un produit de psyllium en boulettes à quatre fois la dose recommandée. Les vétérinaires devraient être au courant que le traitement des coliques chez les chevaux avec des produits de psyllium en boulettes pourrait être associé à une impaction gastrique.(Traduit par Isabelle Vallières).

A novel ultrasonographic assisted technique for desmotomy of the palmar/plantar annular ligament in horses.

OBJECTIVES: To describe an ultrasound assisted technique for desmotomy of the palmar/plantar annular ligament (PAL), determine its efficacy and intraoperative complications. STUDY DESIGN: Cadaveric and in vivo study. ANIMALS: Cadaveric limbs (n = 12), adult horses (n = 4), and clinical cases (n = 2). METHODS: Ultrasound assisted desmotomy of the palmar/plantar annular ligament (UAD-PAL) was performed in cadaveric limbs and in standing horses with the operated limb placed in a distal limb splint. The procedure was performed under general anesthesia and was followed by tenoscopic examination in 2 clinical cases. A hook knife was developed for the procedure. Complete transection was assessed by postmortem dissection (10 forelimbs, 10 hindlimbs) and tenoscopic examination (1 forelimb, 1 hindlimb). Thickness of PAL, surgery time, other intraoperative parameters and complications associated with the procedure were recorded. RESULTS: Complete PAL transection was accomplished in 20/22 limbs. No iatrogenic damage to adjacent intrathecal structures was identified in any case. The instrument was correctly positioned on the first attempt in 19/22 cases. The most common intraoperative complication was inadvertent subcutaneous placement of the instrument (n = 2). Significant thickening of the PAL (3 mm) was present in 1/2 limbs in which complete transection was not achieved. CONCLUSIONS: UAD-PAL with the custom-made hook knife was effective at transecting the PAL with minimal intraoperative complications. The procedure can be performed in standing sedated horses. Another method should be considered in horses with thickened PAL.

Clinical findings and management of 153 horses with large colon sand accumulations.

OBJECTIVE: To determine the influence of radiographic quantification of sand accumulation on the medical versus surgical management of large colon sand accumulations. To compare short- and long-term outcomes and complications associated with medical and surgical management of these horses. STUDY DESIGN: Retrospective. SAMPLE POPULATION: A total of 153 horses. METHODS: Medical records and abdominal radiographs of horses presented for colic between 2004 and 2014 were reviewed. Severity of sand accumulation was quantified by tracing and measuring a region of interest with a commercial software program. Breed, weight, amount of sand, presence of diarrhea at presentation, treatment, and the development of complications were recorded. RESULTS: Records from 153 horses were reviewed. The mean cross-sectional area of sand accumulation was 692.9 cm2 (median = 658.7 cm2 , 84.6-1780.7 cm2 ). Increased accumulation of gas on radiographs and abnormal transrectal examination findings were associated with an increased likelihood of surgery. The most common complication was the development of diarrhea (20.3%) with only 4 (2.6%) horses positive for Salmonella spp. Horses had a favorable prognosis, with 94.8% of horses treated medically and 94.7% of those treated surgically surviving to discharge. CONCLUSION: Increased accumulation of gas on radiographs and transrectal palpation of impaction or intestinal gas distension increase the likelihood of surgery. Both medical and surgical treatments carry a good prognosis. CLINICAL RELEVANCE: The sheer quantity of sand is not a factor when determining surgical intervention. Attention should be paid to the presence of increased gas accumulation on rectal or radiographic examination.

Comparison between the effects of postanesthetic xylazine and dexmedetomidine on characteristics of recovery from sevoflurane anesthesia in horses.

OBJECTIVE: To compare postanesthetic xylazine and dexmedetomidine on recovery characteristics from sevoflurane anesthesia in horses. STUDY DESIGN: Randomized, crossover study. ANIMALS: Six geldings, mean±standard deviation (SD) (range), 17±4 (11-24) years and 527±80 (420-660) kg. METHODS: Horses were anesthetized with sevoflurane for 60 minutes under standardized conditions for a regional limb perfusion study. In recovery, horses were administered either xylazine (200 µg kg-1) or dexmedetomidine (0.875 µg kg-1) intravenously. Recoveries were unassisted and were video-recorded for later evaluation of recovery events and quality by two individuals unaware of treatment allocation. Recovery quality was assessed using a 100 mm visual analog scale (VAS) (0=poor recovery, 100=excellent recovery), the Edinburgh Scoring System (ESS) (0-100; 100=excellent recovery) and the mean attempt interval (MAI) (longer=better). Data are mean±SD. RESULTS: All recovery quality assessments (xylazine and dexmedetomidine, respectively: VAS: 71±21 mm, 84±13 mm; ESS: 65±22, 67±30; MAI: 52±24 minutes, 60±32 minutes) and events (first limb movement: 37±8 minutes, 42±10 minutes; first attempt to lift head: 44±12 minutes, 48±9 minutes; first attempt to sternal posture: 57±28 minutes, 50±7 minutes; number of head bangs: 2.0±3.0, 0.5±0.5; time to first attempt to stand: 72±6 minutes, 78±13 minutes; time to standing: 79±14 minutes, 84±13 minutes) did not differ significantly between treatments (p>0.05). CONCLUSIONS AND CLINICAL RELEVANCE: Recovery characteristics did not differ significantly between postanesthetic xylazine and dexmedetomidine following 1 hour of sevoflurane anesthesia in horses in this study. Further evaluations in more horses and in younger horses are required to confirm these results.

HB Puerta del Sol [HBA1:c.148A>C], HB Valdecilla [HBA2:c.3G>T], HB Gran Vía [HBA2:c.98T>G], HB Macarena [HBA2:c.358C>T] and HB El Retiro [HBA2:c.364_366dupGTG]: description of five new hemoglobinopathies.

BACKGROUND: Structural hemoglobinopathies do not usually have a clinical impact, but they can interfere with the analytical determination of some parameters, such as the glycated hemoglobin in diabetic patients. Thalassemias represent a serious health problem in areas where their incidence is high. The defects in the post-translational modifications produce hyper-unstable hemoglobin that is not detected by most of electrophoretic or chromatographic methods that are available so far. METHODS: We studied seven patients who belong to six unrelated families. The first two families were studied because they had peak abnormal hemoglobin (Hb) during routine analytical assays. The other four families were studied because they had microcytosis and hypochromia with normal HbA2 and HbF without iron deficiency. HbA2 and F quantification and abnormal Hb separation were performed by chromatographic and electrophoretic methods. The molecular characterization was performed using specific sequencing. RESULTS: The Hb Puerta del Sol presents electrophoretic mobility and elution in HPLC that is different from HbA and similar to HbS. The electrophoretic and chromatographic profiles of the four other variants are normal and do not show any anomalies, and their identification was only possible with sequencing. CONCLUSIONS: Some variants, such as Hb Valdecilla, Hb Gran Vía, Hb Macarena and Hb El Retiro, have significant clinical impact when they are associated with other forms of α-thalassemia, which could lead to more serious forms of this group of pathologies as for HbH disease. Therefore, it is important to maintain an adequate program for screening these diseases in countries where the prevalence is high to prevent the occurrence of severe forms.