Mucopolysaccharidosis in a cat with arylsulfatase B deficiency: a model of Maroteaux-Lamy syndrome.

A Siamese cat that presented clinical signs similar to those seen in humans with mucopolysaccharidoses was studied. The animal excreted increased amounts of polymeric glycosaminoglycans in the urine, consisting almost entirely of dermatan sulfate. Electron microscopy of circulating polymorphonuclear leukocytes revealed the presence of many membrane-bound lamellar inclusion bodies. Sulfate incorporation studies with cultured skin fibroblasts indicated defective glycosaminoglycan degradation. These cells showed a deficiency in arylsulfatase B activity. The disorder appears similar or identical to the Maroteaux-Lamy syndrome described in humans.

Polyclonal activation of canine B lymphocytes evaluated by a protein A reverse hemolytic plaque assay.

This paper describes the optimal culture and assay conditions for the polyclonal activation of canine lymphocytes with pokeweed mitogen and the quantitation of immunoglobulin secreting plaque-forming cells (PFC) using a staphylococcal protein A-reverse hemolytic plaque assay. The assay permits the quantitation of total immunoglobulin secreting PFC as well as class-specific immunoglobulin secreting PFC. On the optimal day of culture, a mean of 176 IgA PFC/10(6), 575 IgM PFC/10(6), 1276 IgG PFC/10(6), and 2158 total PFC/10(6) cells were generated following polyclonal activation. This study provides a simple and reproducible assay for the delineation of the immunoregulatory mechanisms involved in the differentiation of canine B lymphocytes.

Potentiation of the canine lymphocyte blastogenic response by indomethacin.

Cultivation of normal canine peripheral blood lymphocytes in the presence of indomethacin, a potent prostaglandin synthetase inhibitor, produced a consistent potentiation of their in vitro response to mitogenic stimulation as compared to cultures without indomethacin. The enhancing effect of indomethacin increased with decreasing concentrations of mitogens. Indomethacin had to be added to the cultures during the first four hours for the potentiating effect to be observed. These data suggest that prostaglandins may play an important role in the regulation of the dog's immune response.

Fatty acid relationship in an aquatic food chain.

The relationships amongst the fatty acids of the lipids from members of a model aquatic food chain were examined. The basic pattern of the fatty acids in the members, algae-brine shrimp-hydra, originated in the phytoplankton. Fatty acids in the neutral lipids of adult brine shrimp,Artemia salina, closely resembled dietary, or algal, fatty acids, whereas the phospholipid acids differed considerably from those in the algae. Fatty acids from the total lipids ofHydra pseudoligactis fed brine shimp nauplii also resembled the dietary acids, but more C(20) polyunsaturates and fewer C(18) unsaturated acids were present in those raised at 10C than were found at 20C.

Sry-negative XX sex reversal in a family of Norwegian Elkhounds.

Two related female Norwegian Elkhounds were evaluated at 6 and 8 months of age for enlarged clitori. Both had a 78 XX karyotype. Histology of their internal reproductive tracts demonstrated 1 to be an XX true hermaphrodite with bilateral ovotestes and the other to be an XX male with bilateral aspermatogenic testes. Polymerase chain reaction-based tests of genomic DNA showed that both dogs lacked Sry, the testis-determining gene. Pedigree analysis was consistent with an autosomal recessive mode of inheritance, as has been reported in the American Cocker Spaniel and the German Shorthaired Pointer. This is the 1st reported case of familial Sry-negative XX sex reversal in the Norwegian Elkhound. A summary of 34 previously unreported cases of dogs with masculinized external genitalia and a normal 78 XX karyotype seen from 1980 to 1997 is given.

Free amino acids in the plasma and urine of dogs from birth to senescence.

Plasma and urinary amino acid concentrations were determined from a cross-sectional sample of dogs of all ages. Reference or base line values for these components were established for five different age groups. A general decrease in urinary excretion of amino acids for each gram of creatinine was found during the developmental period. This decrease was not correlated to changes in plasma concentrations, which varied for the individual amino acids. The decrease did appear to be related to the functional maturation of the kidney. Several methylated basic amino acids were identified in the urine. Of these, NG, NG- and NG,N'G-dimethylarginine, and trimethyllysine had not been previously reported as constituents of dig urine, but are major components of the urinary amino acids, expecially in newborn animals. The 3-methylhistidine is also excreted in large amounts by dogs and measurable quantities of this compound appear in the plasma. These findings suggest an active metabolic function for methylated amino acids in the dog.

Transfer of colostral antibodies from queens to their kittens.

OBJECTIVE: To examine systemic immunity in kittens, including transfer of maternal immunoglobulins from the queen to kittens, and subsequent decay of passively obtained immunoglobulins. ANIMALS: 6 healthy queens and their 46 kittens. PROCEDURE: Immunoglobulin concentrations were measured in serum, colostrum, and milk of queens and in their kittens' sera. Decay rate constants and half-lives of maternally derived immunoglobulins were determined. To determine intestinal absorption, foreign IgG was given to kittens at 6- to 8-hour intervals after birth, and bovine IgM was given to kittens at birth. RESULTS: Immunoglobulin concentrations of milk and colostrum did not differ significantly after removal of milk fat. Mean IgG concentration was higher in colostrum/ milk, whereas mean IgA and IgM concentrations were lower than those in the queens' serum. No IgG or IgA was detected in any of the precolostral serum samples obtained from kittens. Small amounts of IgM were present in the sera from 5 kittens at birth. Transferred IgG and IgA decreased rapidly with half-lives of 4.4 +/- 3.57 and 1.93 +/- 1.94 days, respectively. Serum IgM concentration increased irregularly during the first week of life, followed by a steady increase. Foreign IgG given up to 12 hours after birth was detected in kittens' serum, whereas IgG given at or after 16 hours was not found in any kitten's serum. CONCLUSIONS: Milk and colostral immunoglobulin concentrations did not differ significantly. The half-lives of maternally derived IgG and IgA in kittens were shorter than those reported in dogs. IgG given at or after 16 hours of life was not absorbed by neonatal kittens. CLINICAL RELEVANCE: Queen's milk obtained anytime during lactation may be used as a replacement for colostrum as a source of antibodies for neonatal kittens. Kittens at risk for neonatal isoerythrolysis must only be removed from the queens during the first day of life.

Postnatal development of renal tubular amino acid reabsorption in canine pups.

The renal clearance of amino acids in canine pups 5 days and 3, 8, and 12 weeks of age was studied. The 5-day-old pups had incomplete reabsorption of most amino acids. At 3 weeks, fractional reabsorption of 16 of 21 amino acids was complete; between 3 and 8 weeks, an adult pattern of reabsorption was present. Seemingly, a rapid development of amino acid transport precedes anatomic development or functional maturation of other transport systems.

Nitrosonapthol test for screening of small intestinal diarrheal disease in the dog.

The nitrosonapthol test, which qualitatively measures urinary excretion of 4-hydroxyphenylacetic acid and related compounds, was evaluated as a screening test for small intestinal disease in 60 dogs with chronic diarrhea. Test results were positive in 8 of 13 dogs with pancreatic exocrine insufficiency, in 8 of 12 dogs with small intestinal malabsorption, in 2 of 17 dogs with colitis, and in all of 14 dogs with normal gut function and in which the specific cause of diarrhea was not determined. Test results were negative in 4 dogs with functional (stress-induced) diarrhea and in 25 clinically normal dogs. Thus, the test results were positive in 30 of 39 (77%) dogs with confirmed small intestinal or pancreatic disease or in which small intestinal disease was the most likely diagnosis, and in only 2 of 21 (9.5%) dogs with large intestinal disease (P less than 0.001). In human medicine, positive test results have been associated with bacterial overgrowth in the small intestine. Accordingly, antibiotics were administered orally to 8 of the dogs with diarrhea of undetermined cause. In all treated dogs, antibiotic therapy resulted in improvement in fecal consistency, decreased frequency of defecation, and a change from a positive to a less strongly positive or negative reaction to the nitrosonapthol test. These findings suggested that the nitrosonapthol test is useful in the discrimination of small intestinal from large intestinal diarrhea and as a guide to the use of oral antibiotic therapy in dogs with chronic diarrhea of undetermined cause.

Myocarditis of probable viral origin in pups of weaning age.

Myocarditis in 4- to 8-week-old pups from 10 litters was characterized by sudden death. Histopathologic findings included mononuclear cellular infiltration and interstitial fibrosis in the myocardium of the left ventricle. Basophilic intranuclear inclusion bodies were seen in myocardial cells in 4 of 18 pups necropsied, suggesting a viral origin of the disease. Other pathologic changes were variable, but all were attributable to cardiac failure. Of 8 surviving pups examined, 7 had evidence of cardiac failure, including pulmonary edema, cardiomegaly, and cardiac arrhythmias.

Alpha-mannosidosis in a Persian cat.

Alpha-mannosidosis, an inherited enzyme-deficiency disease, caused growth retardation, hepatomegaly, ocular abnormalities, and neurologic dysfunction in a Persian cat. Because of the clinical progression of this autosomal recessive, lysosomal storage disorder, the cat was euthanatized at 5 months of age.

Pseudoachondroplastic dysplasia in miniature poodles: clinical, radiologic, and pathologic features.

The clinical, radiologic, and pathologic features of pseudoachondroplastic dysplasia were studied in 13 affected Miniature Poodles. The disease became evident when pups were about 3 weeks old. Normal-appearing and affected pups were in the same litter, and both sexes were affected. Abnormal locomotion with abducted hindlimbs and flattened rib cages were accompanied by short, bent limbs and enlarged joints. Radiographically, areas of stippling and patchy densities were evident in the epiphyses. Histologically, these densities were identified as areas of bone associated with retarded ossification. After growth is completed, the epiphyses, ossify, but the bones are shortened and deformed. General health appears normal, except for restricted locomotion due to secondary arthritis and difficult breathing in dogs with a deformed thorax.

Lethal acrodermatitis in bull terriers.

A lethal syndrome characterized clinically by growth retardation, progressive acrodermatitis, chronic pyoderma and paronychia, diarrhea, pneumonia, and abnormal behavior was observed in 17 related Bull Terrier pups. Median survival time was 7 months. Laboratory evaluation revealed non-degenerative neutrophilia, consistently low activities of serum alkaline phosphatase and alanine transaminase, and frequently, hypercholesterolemia. Lymphocyte blastogenic responses were decreased and there was dysgammaglobulinemia in pups in which quantitative studies of immunoglobulins were made. The mean of plasma zinc concentrations in 5 affected pups was significantly lower than the mean of age- and breed-matched controls. Pathologic findings included parakeratosis, hyperkeratosis, and superficial bacterial infections of the skin. There was severe reduction of lymphocytes in T-lymphocyte areas of lymphoid tissue. Bronchopneumonia and dilatation of the cerebral ventricles were found in most affected pups. Family studies indicated that the syndrome is inherited as an autosomal recessive trait. In spite of its similarities to lethal trait A46 in Black Pied Danish cattle and acrodermatitis enteropathica in man, oral or parenteral treatment with zinc failed to ameliorate the clinical signs of the syndrome.

Mucopolysaccharidosis in a cat.

A young adult female Siamese cat born of a mother-son mating was referred because of dwarfism, facial abnormalities, severe skeletal deformities, multifocal neurologic deficits, and retinal atrophy. Cats of similar appearance had been observed in a previous litter of the same parents. Metachromatic inclusion bodies were demonstrated in circulating leukocytes. The urine contained a high concentration of mucopolysaccharide, as detected by the toluidine blue spot test. The uronic acid content of the cetylpyridinium chloride-precipitable mucopolysaccharide in the urine was 17 times greater than that in the urine from a control cat of the same age and breed.

Mucopolysaccharidosis in a domestic short-haired cat--a disease distinct from that seen in the Siamese cat.

A 10-month-old male domestic short-haired cat was examined because of progressive lameness, a broad face with depressed nasal bridge, small ears, corneal clouding, and multiple bone dysplasia. The cat excreted excessive amounts of glycosaminoglycan (a component of connective tissue) in its urine and had evidence of lysosomal storage of glycosaminoglycans in fibroblasts and neurons. Activity of alpha-L-iduronidase, a lysosomal enzyme involved in glycosaminoglycan degradation, was deficient in cultured fibroblasts and leukocytes. The mucopolysaccharidosis was distinct from that seen in Siamese cats in terms of the pathologic changes and the specific enzyme deficiency.

Short-limbed dwarfism and ocular defects in the Samoyed dog.

A syndrome of short-limbed dwarfism and ocular defects was found in Samoyed dogs. The most prominent abnormalities were small stature and valgus deformity of the carpi. Radiographic evidence of retarded growth at the distal ulnar physis was apparent by 12 weeks of age. Ocular defects included cataracts and retinal detachment. Family studies and limited breeding experiments were consistent with an autosomal recessive mode of inheritance.

Spinal cord compression and hindlimb paresis in cats with mucopolysaccharidosis VI.

Six cats with mucopolysaccharidosis VI had hindlimb paresis and other clinical signs associated with compression of the thoracolumbar spinal cord. In 5 cats, the neurologic abnormality progressed over 2 to 4 weeks to loss of thoracolumbar spinal cord function. In 1 cat, the hindlimb paresis remained stable for 18 months. In the cats with progressive worsening of hindlimb function, the abnormality was caused by compression of the spinal cord from proliferation of bony tissue in the thoracolumbar region. In all affected cats, the compression occurred from T12 to L2. In 1 cat, an attempt to relieve the clinical signs by surgery was unsuccessful.

Myocarditis induced by parvoviral infection in weanling pups in the United States.

In a nationwide survey from January 1978 through September 1979, acute parvovirus-induced myocarditis was identified in 76 of 147 (52%) pups in 19 litters. The affected pups were from 12 states. Birth of all pups registered with the American Kennel Club during this period increased during the spring months, peaking in May, which was correlated in 4 to 8 weeks with the greatest number of cases in the study. Serologic data available for 4 dams, for 6 of their surviving pups, and for 3 contact pups indicated that all had been exposed to parvovirus. In 2 kennels, cases of acute myocarditis were preceded within 2 months by cases of severe gastroenteritis in older litters on the same premises.