Shar-Pei autoinflammatory disease with skin necrosis due to neutrophilic vasculitis: a case report.

A 4-month-old female Shar-pei dog was admitted with apathy, anorexia, and vomiting. The patient had an appropriate vaccination protocol, with the last vaccine administered 2.5 weeks prior to the onset of clinical signs. Physical examination revealed tachycardia, fever and swelling of the tibiotarsal joints. Several diagnostic tests including complete blood cell count, biochemistry profile, urinalysis, thoracic radiographs, hind limbs radiographs, abdominal ultrasound, and infectious diseases tests, were conducted to determine the underlying cause. Shar-Pei Auto-inflammatory Disease (SPAID) was diagnosed. Additionally, the patient developed skin necrosis in the inner aspect of the tibiotarsal joints as a complication. A skin biopsy revealed cutaneous vasculopathy causing degeneration, abrupt ulceration, and ischemic necrosis with intense neutrophilic inflammation of the dermis and subcutis. Moreover, a hospital-acquired infection was identified by skin culture. Debridement of the necrotic skin was performed, and due to its' severity and extent, the wound was closed by secondary intention. A diagnostic protocol and the therapeutic dose of acetylsalicylic acid, which led to clinical improvement, are recommended here. The patient has continued to present episodic manifestations of SPAID mainly fever and swelling of the tibiotarsal joints, but there has been no recurrence of necrosis or other cutaneous lesion in the last two years.

Case report: Radiofrequency-induced thermal burn injury in a dog after magnetic resonance imaging.

A 10-year-old male Shar-Pei was referred for lethargy and proprioceptive deficits of the left thoracic limb. An magnetic resonance imaging (MRI) examination of the cervical spinal column and the brain was performed. The MRI examination of the brain was normal. A left-sided C3-C4 intervertebral disc extrusion with spinal cord compression was diagnosed. Medical treatment was elected. Within a week after the MRI examination, the dog presented with deep partial-thickness skin burn wounds in both axillae. Since the specific absorption rate had not exceeded the safety limits during any of the scans and no other procedures or circumstances were identified that could possibly have resulted in burn injuries, the thermal burn injuries were diagnosed as radiofrequency (RF) burns. The wounds healed by secondary intent over the next month. RF burns are the most reported complication in humans undergoing MRI but have not been reported in veterinary patients. Clinicians and technicians should consider the potential risk for RF burns in veterinary patients and take precautions regarding positioning of the patient and take notice of any signs of burn injury when performing follow-up examinations.

Case report: Sublingual mucinosis in a dog.

A 11-month-old male intact Shar-Pei (26. 5 kg) was presented for a bilateral sublingual swelling of 4 months duration. The exploration of the oral cavity highlighted the presence of bilateral sublingual swellings, primarily consistent with bilateral ranula. The bilateral disease was treated with two subsequent surgeries 4 weeks apart. During the surgery, after removing an elliptical portion of the mucosa of the sublingual swelling, the presence of gelatinous tissue was visualized, and no saliva was present. The result of histological exam was oral mucinosis. At the subsequent follow-up the dog was in excellent conditions, without any symptoms. 1 month after the last operation, the dog underwent a visit in sedation to better evaluate the oral cavity. Both surgical sites were well-healed and without the presence of relapses. Upon 8 months follow-up the patient remained free of disease. This is the first reported case of oral mucinosis in sublingual mucosa in dogs. In this case the surgical treatment was curative.

Surgical management of bilateral, upper and lower eyelid entropion in 27 Shar Pei dogs, using the Stades forced granulation procedure of the upper eyelid only.

Aims: To evaluate the outcome of surgical management of entropion of the upper and lower eyelids in Shar Pei dogs which had previously undergone temporary palpebral tacking, using the Stades forced granulation procedure of the upper eyelid only.Methods: Medical records were retrospectively searched to identify Shar Pei dogs referred for bilateral entropion of both the lower and upper eyelids, and that were treated with the forced granulation procedure of the upper eyelid only. Dogs were included if they had previously undergone unsuccessful temporary palpebral tacking and had at least three follow-up examinations in the 30 days following surgery.Results: Twenty-seven Shar Pei dogs with a median age of 7.9 (min 4, max 24) months were included in the study. Before surgery, all dogs showed signs of severe ocular discomfort with bilateral keratitis and visual deficit due to blepharospasm, enophthalmos and protrusion of the third eyelid. In 13/54 eyes, keratitis was associated with a corneal ulcer. When re-examined 4 weeks after surgery, correction of the upper eyelid entropion and associated trichiasis resolved ocular signs in 50/54 eyes. Mild bilateral lower entropion remained in two dogs postoperatively, which underwent revision surgery with the Hotz-Celsus technique. There were no cases of long-term recurrence of entropion or ocular irritation in the 38 eyes (19 dogs) which were re-examined 1 year after surgery.Conclusion and clinical relevance: The forced granulation procedure performed on the upper eyelid only was effective for correction of entropion in the Shar Pei dogs included in this study. In our experience, it is preferable to operate on the upper eyelid alone, rather than attempting to correct upper and lower entropion during the same surgical operation. The dog can then be reassessed a few weeks later to determine whether the lower entropion is anatomical or secondary to the severe blepharospasm resulting from the painful ocular irritation.

Shar Pei Larynx: Supraglottic and Postcricoid Mucosal Redundancy and Its Association With Medical Comorbidities.

OBJECTIVES:: The aim of this study is to describe a clinical entity the authors term "Shar Pei larynx," characterized by redundant supraglottic and postcricoid mucosa that the authors hypothesize coexists in patients with obstructive sleep apnea, laryngopharyngeal reflux, and obesity. By exploring this hypothesis, the authors hope to set the foundation for future research with the goal of identifying whether Shar Pei larynx is a marker for untreated sleep apnea or other diseases. STUDY DESIGN:: Retrospective chart review. SETTING:: Two tertiary care academic institutions. METHODS:: Data were collected from a 5-year period by querying for patients described to have "Shar Pei larynx" or "posterior supraglottic and/or postcricoid mucosal redundancy" on laryngoscopic findings. Relevant demographic and clinical characteristics were analyzed, with a focus on associations with obesity, sleep apnea, and laryngopharyngeal reflux. RESULTS:: Thirty-two patients were identified with physical findings consistent with Shar Pei larynx. Twenty-six patients (81.3%) were obese; 16 (50%) were morbidly obese. Twenty-two patients (68.8%) either had an existing diagnosis of obstructive sleep apnea or were diagnosed on polysomnography performed after initial evaluation. Sixteen patients (50%) had type 2 diabetes mellitus, and 87.5% of these patients were obese. Twenty-eight patients (87.5%) noted histories of reflux, with a median reflux symptom index of 27 of 45. Five patients underwent procedures to reduce mucosal redundancy related to Shar Pei larynx. CONCLUSIONS:: This pilot study confirms that the majority of patients diagnosed with Shar Pei larynx also had diagnoses of obesity, obstructive sleep apnea, and reflux disease. The demonstrated association is strong enough to warrant further study.

Modified brow suspension technique for the treatment of pseudoptosis due to redundant frontal skin in the dog: a retrospective study of 25 cases.

PURPOSE: To describe the outcome of modified brow suspension technique for the treatment of upper eyelid pseudoptosis in canine patients with redundant frontal skin folds. METHODS: Medical records from 1999 to 2015 were retrospectively reviewed. Only dogs that underwent modified brow suspension for treating bilateral pseudoptosis were included in the study. Recorded data included breed, age, gender, main ophthalmic complaint, ocular findings, suture material used for brow suspension, number of slings placed, postoperative treatment, complications, follow-up time, and outcome. RESULTS: Twenty-five adult dogs of different gender and breed fulfilled the inclusion criteria, with the Shar-Pei being overrepresented (19/25;76%). The main complaint at first presentation was mucopurulent discharge (10/25;40%) and blepharospasm (10/25;40%). Concurrent corneal disease was diagnosed in 47 eyes (47/50;94%). Monofilament polyamide was the most frequently used suture material (46/50;92%). Number of slings placed varied, with one in two eyes (4%), two in 28 eyes (56%), three in 17 eyes (34%), and four in three eyes (6%). Complications were seen in three eyes (6%): suture rupture (one eye; 2%) and skin abscess (two eyes; 4%). Mean follow-up time was 17.6 months (1-84 months), with no cases of fold dermatitis or dermatologic disease observed within this period. Good esthetic and functional outcomes were obtained in 47 eyes at first intervention (97%) and in all patients after re-intervention. CONCLUSIONS: The modified brow suspension technique described offers a surgical alternative for dogs with pseudoptosis secondary to redundant frontal skin folds that potentially reduces anesthetic time and facial changes.

Whole genome sequencing identifies missense mutation in MTBP in Shar-Pei affected with Autoinflammatory Disease (SPAID).

BACKGROUND: Autoinflammatory diseases in dogs are characterized by complex disease processes with varying clinical signs. In Shar-Pei, signs of inflammation including fever and arthritis are known to be related with a breed-specific predisposition for Shar-Pei Autoinflammatory Disease (SPAID). RESULTS: Clinical and histopathological examinations of two severely SPAID-affected Shar-Pei revealed signs of inflammation including fever, arthritis, and perivascular and diffuse dermatitis in both dogs. A multifocal accumulation of amyloid in different organs was found in one SPAID-affected case. Whole genome sequencing resulted in 37 variants, which were homozygous mutant private mutations in SPAID-affected Shar-Pei. Nine SNVs with predicted damaging effects and three INDELs were further investigated in 102 Shar-Pei affected with SPAID, 62 unaffected Shar-Pei and 162 controls from 11 different dog breeds. The results showed the missense variant MTBP:g.19383758G > A in MTBP to be highly associated with SPAID in Shar-Pei. In the region of this gene a large ROH (runs of homozygosity) region could be detected exclusively in the two investigated SPAID-affected Shar-Pei compared to control dog breeds. No further SPAID-associated variant with predicted high or moderate effects could be found in genes identified in ROH regions. This MTBP variant was predicted to affect the MDN2-binding protein domain and consequently promote proinflammatory reactions. In the investigated group of Shar-Pei older than six years all dogs with the mutant genotype A/A were SPAID-affected whereas SPAID-unaffected dogs harbored the homozygous wildtype (G/G). Shar-Pei with a heterozygous genotype (G/A) were shown to have a 2.13-fold higher risk for disease development, which gave evidence for an incomplete dominant mode of inheritance. CONCLUSIONS: The results of this study give strong evidence for a variant in MTBP related with proinflammatory processes via MTBP-MDM2 pathway. Thus, these results enable a reliable detection of SPAID in Shar-Pei dogs.

Inflammatory, immunological, and intestinal disease biomarkers in Chinese Shar-Pei dogs with marked hypocobalaminemia.

Chinese Shar-Pei dogs have a high prevalence of hypocobalaminemia and are commonly presented with clinical signs suggestive of severe and long-standing gastrointestinal disease such as diarrhea, vomiting, and/or weight loss. The aim of the current study was to evaluate serum concentrations of inflammatory markers, markers for intestinal disease, and immunological markers in Shar-Peis with hypocobalaminemia or normocobalaminemia (serum cobalamin concentrations within the reference interval). Serum samples from Shar-Peis were collected from various parts of the United States. Serum concentrations of inflammatory markers (i.e., C-reactive protein [CRP], calprotectin [CP], and S100A12), hyaluronic acid (HA, a marker for cutaneous mucinosis), and analytes commonly altered in chronic intestinal diseases (i.e., albumin, zinc, alpha1-proteinease inhibitor [α1PI], immunoglobulin [Ig]A, and IgM) were compared between Shar-Peis with hypocobalaminemia and Shar-Peis with normocobalaminemia. Serum concentrations of CRP, CP, S100A12, HA, zinc, and cα1-PI concentrations did not differ between hypocobalaminemic and normocobalaminemic Shar-Peis (P > 0.05). Serum concentrations of albumin were significantly lower in hypocobalaminemic Shar-Peis (median: 2.5 g/dl) than in normocobalaminemic Shar-Peis (median: 2.9 g/dl; P < 0.0001). Higher serum IgA concentrations and lower serum IgM concentrations were observed in hypocobalaminemic Shar-Peis (median: 1.7 g/l and 0.8 g/l, respectively) than in normocobalaminemic Shar-Peis (median: 0.7 g/l and 1.9 g/l, respectively; both P < 0.0001). In conclusion, no difference was found in serum concentrations of CRP, CP, S100A12, and HA between hypocobalaminemic and normocobalaminemic Shar-Peis whereas some differences were observed in analytes (e.g., albumin, IgA, and IgM) that may be altered in patients with chronic enteropathies.

Serum homocysteine and methylmalonic acid concentrations in Chinese Shar-Pei dogs with cobalamin deficiency.

Cobalamin deficiency is suspected to be hereditary in Chinese Shar-Pei dogs (Shar-Peis), and inherited causes of cobalamin deficiency may affect the cellular processing of cobalamin. In humans, a defect of the two main cobalamin-dependent intracellular enzymes (i.e., methionine synthase and methylmalonyl-CoA mutase) may lead to hyperhomocysteinemia and hypermethylmalonic acidemia. The aim of this retrospective study was to evaluate serum homocysteine (HCY) and methylmalonic acid (MMA) concentrations in cobalamin-deficient Shar-Peis and dogs of six other breeds. Serum samples (n=297) from cobalamin-deficient dogs (Shar-Peis, German Shepherd dogs, Labrador Retrievers, Yorkshire Terriers, Boxers, Cocker Spaniels, and Beagles) were analyzed for serum HCY and MMA concentrations. A Fisher's exact test was used to evaluate if cobalamin deficiency in Shar-Peis is associated with hyperhomocysteinemia. Serum HCY and MMA concentrations were higher in cobalamin-deficient Shar-Peis compared to cobalamin-deficient dogs of the six other breeds (P<0.0001). Hyperhomocysteinemia was associated with cobalamin deficiency in Shar-Peis (P=0.009). In addition, serum HCY and MMA concentrations did not differ between cobalamin-deficient German Shepherd dogs with and without exocrine pancreatic insufficiency (EPI), a potential cause of secondary cobalamin deficiency. These findings suggest that the function of the two intracellular cobalamin-dependent enzymes is impaired in Shar-Peis with cobalamin deficiency.

Osteocondrite dissecante do côndilo femoral lateral em cão shar-pei: relato de caso / Osteochondritis dissecans in the femoral lateral condylus in the shar-pei dog: case report / Osteocondritis disecante del cóndilo femoral lateral en perro shar-pei: relato de caso

Descreve-se o tratamento cirúrgico de osteocondrite dissecante do côndilo femoral lateral, em um cão macho adulto da raça Shar-Pei, com acesso para-patelar lateral ao joelho. O diagnóstico foi realizado por meio de exame ortopédico e radiográfico. Como tratamento, realizou-se artrotomia e condiloplastia femoral lateral, removendo retalho cartilaginoso e expondo o osso subcondral. Foram perfurados orifícios, com a finalidade de acelerar o processo cicatricial. No período pós-operatório houve restrição de exercícios durante 10 dias, com o retorno gradual às atividades físicas. Indicou-se o uso de tramadol e cetoprofeno durante sete dias, e sulfato de condroitina por 100 dias. Quarenta dias após a cirurgia, o paciente apresentou ausência de dor e claudicação, retornando à ambulação normal.

This paper reports the surgical treatment of osteochondritis dissecans in the femoral lateral condylus in a male adult Shar-Pei dog, with lateral parapatellar access to the knee. Diagnosis was made by physical and radiographic examination. The treatment was arthrotomy and lateral femoral condylar plastia, removing a cartilaginous flap and exposing the subchondral bone, in which there were performed punctures in order to accelerate the healing process. Exercises were restricted during the first 10 days of the post-op period, and then a gradual return to physical activities was recommended. Tramadol and ketoprofen were prescribed for seven days, and chondroitin sulphate for 100 days. Forty days after the surgery the patient showed absence of pain or lameness, returning to normal ambulation.

Se relata el tratamiento quirúrgico de osteocondritis disecante del cóndilo femoral lateral, en un perro Shar-Pei macho adulto, con acceso pararotuliano lateral a la rodilla. El diagnóstico fue realizado por medio de examen ortopédico y radiográfico. Como tratamiento, se realizó artrotomía y condiloplastía femoral lateral, removiéndose colgajo cartilaginoso y exponiendo el hueso subcondral. Se realizaron perforaciones, con objetivo de acelerar el proceso de cicatrización. En el periodo post-operatorio se indicó restricción a los ejercicios por 10 días, y después un retorno gradual a las actividades físicas. Se prescribió tramadol y ketoprofeno durante siete días, y sulfato de condroitina por 100 días. Cuarenta días después de la intervención el paciente no más demostraba dolor y claudicación, retornando a la ambulación normal.