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Biomedical subjects

A Berman

Publications and source records attributed to A Berman.

At least 19 recordsLinked to original sources

The urological manifestations of chronic granulomatous disease.

Chronic granulomatous disease is a rare disorder of phagocytic cell oxidative metabolism. Patients have recurrent infections with catalase positive organisms and granulomatous lesions throughout the body. The genitourinary tract can be an occult site of involvement. We reviewed the prevalence of urological abnormalities in 60 patients with chronic granulomatous disease. Chart review revealed that 23 of the 60 patients (38%) had urological manifestations. Seven patients had 10 ureteral strictures. Treatment of the granulomatous strictures with steroids caused at least a temporary decrease in size or a resolution of the granulomas and strictures with steroids caused at least a temporary decrease in size or a resolution of the granulomas and relief of the obstruction. Three patients had bladder granulomas and 1 had a urethral stricture. A total of 12 patients had urinary tract infections and 7 had altered renal function. Ten patients had other manifestations of genitourinary disease. Abnormalities in the urinary tract are relatively frequent. Risk factors include X-linked disease and low percentile weight. Genitourinary manifestations can cause significant morbidity in chronic granulomatous disease patients. A variety of therapies, including judicious use of steroids and gamma-interferon, are proving to be useful in the treatment of this disorder and in some cases they may offer a nonoperative alternative for relief of chronic granulomatous obstructive disease in these patients.

Female

Chronic idiopathic hyperphosphatasia. Report of a case treated with pamidronate and a review of the literature.

We describe the case of a 38-year-old woman with 24 years of progressive skeletal deformities. Radiologic survey showed evidence of generalized skeletal involvement. She presented with secondary osteoarthritis, and her hands showed Heberden's and Bouchard's nodes. Light microscopic examination showed many reversed lines in the trabeculae and increased bone resorption and bone forming areas. On electron microscopic study there were intranuclear inclusions in the osteoclasts. To our knowledge, she is the oldest patient described in the English literature, and the first treated with (3-amino-1-hydroxypropilidene)-1,1 biphosphonate (Pamidronate). She showed a decrease in serum alkaline phosphatase, urinary hydroxyproline. Clinical variables showed good response to medication.

Adult

Water, sodium, potassium, and chlorine metabolism of dairy cows at the onset of lactation in hot weather.

Water, Na, K, and Cl balances, blood plasma composition, and urine and fecal outputs were studied in 5 high yielding cows (greater than 30 kg/d milk) at 2 wk prepartum and at 2 and 7 wk postpartum during the summer in Israel. Cows were fed complete diets with electrolyte content exceeding dietary recommendations. Plasma volume, as assessed by hematocrit changes, was greater postpartum, probably due to increased heat load and water turnover. Milk secretion markedly increased electrolyte output, which was compensated for only partially by increased intake. This was associated with marked reduction of electrolyte losses in excreta, particularly that of Na and Cl. On the basis of urea excreted in the urine, it seems that the current practice of abruptly increasing protein content of the diet at the onset of lactation might reduce the efficiency of dietary protein utilization, compared with efficiency of protein utilization at a later stage of lactation. The need to excrete excessive N also adversely affected the water and electrolyte balances. At initiation of lactation, when DMI is still limited and hot weather obstructs its rapid increase, the current recommendations for electrolytes as a percentage of the ration do not meet the needs.

Animals

Rheumatic manifestations in populations at risk for HIV infection: the added effect of HIV.

We assessed the frequency and variability of rheumatologic manifestations in 2 populations with similar risk factors for human immunodeficiency virus (HIV) infection, of which only one was affected. We studied 2 populations at risk for HIV infection (homosexual and intravenous drug users). Group A: 89 individuals carried the virus; and Group B: 80 individuals were HIV negative. In Group A (HIV+) 66.1% had rheumatic manifestations including arthralgias, Reiter's syndrome, arthritis, enthesitis, psoriatic arthritis, Sjögren's syndrome, myopathy, septic arthritis, and lupus-like illness. In contrast, in Group B (HIV-) only 2 had arthralgias, 2 Reiter's syndrome, and 1 rheumatoid arthritis. In summary, rheumatic complaints are common in patients with HIV, and HIV positivity confers an increased susceptibility in populations with similar risk factors for HIV infection.

Adolescent

Localization of the von Hippel-Lindau disease gene to a small region of chromosome 3.

We studied 25 families with von Hippel-Lindau disease (VHL) to locate VHL more precisely on chromosome 3. We found that VHL was linked to RAF1, confirming previous observations, and to two polymorphic DNA markers, D3S18 and D3S191. Multipoint linkage analysis indicated that the most likely location for VHL was in the interval between RAF1 and D3S18. D3S18 was located at 3p26. Genetic heterogeneity was not detected in this panel of von Hippel-Lindau disease families. The polymorphic markers RAF1, D3S18, and D3S191 should be useful in identifying asymptomatic gene carriers in VHL families and in guiding efforts at gene isolation.

Chromosome Mapping

Preparative cytoreductive surgery in patients with metastatic renal cell carcinoma treated with adoptive immunotherapy with interleukin-2 or interleukin-2 plus lymphokine activated killer cells.

A total of 63 patients with metastatic renal cell carcinoma with the primary kidney tumor in place was accepted as candidates for immunotherapy at the Surgery Branch of the National Cancer Institute. Of the 63 patients 54 underwent nephrectomy and 9 were treated with the primary kidney tumor in place. Many of the patients underwent associated procedures, such as regional lymphadenectomy (11), venacavotomy with extraction of tumor thrombus (9), hepatic resection (2), pulmonary wedge resection (2), cholecystectomy (2), splenectomy (2), distal pancreatectomy (1), omentectomy (1) and contralateral adrenalectomy (1). Of the 54 patients 20 were not able to enter therapy because of tumor-related (17) or other medical (3) reasons that developed between the operation and therapy, while 34 were able to receive immunotherapy postoperatively. The 20 patients who were treated with either high dose interleukin-2 or interleukin-2 plus lymphokine activated killer cells soon postoperatively (mean 2.1 months) were able to tolerate roughly the same amount of interleukin-2 as the 74 who had undergone nephrectomy before referral to our institute and who were treated for a mean of 22 months after nephrectomy. Further studies, including a prospective, randomized trial, will be required to define the role of nephrectomy in patients with advanced renal cell carcinoma before treatment with interleukin-2 based immunotherapies.

Carcinoma, Renal Cell

Optic nerve involvement associated with the multiple evanescent white-dot syndrome.

Five patients presenting with signs and symptoms of primary optic nerve disease were, after careful examination, diagnosed as having the multiple evanescent white-dot syndrome. Optic nerve findings in these patients included disc edema, central visual loss, enlarged blind spots, and other optic nerve field defects. The subtlety of the white spots in some cases made the diagnosis difficult. Multiple evanescent white-dot syndrome should be considered in the differential diagnosis of young, healthy patients who present with unilateral or bilateral optic nerve dysfunction.

Adult

HIV associated arthropathy: HIV antigen demonstration in the synovial membrane.

Light and electron microscopic studies were performed on the synovial membranes of 5 patients with HIV associated arthropathy. An immunoperoxidase technique with the use of monoclonal antibodies against CD4, CD8, B and DR lymphocytes, and HIV p24 antigen was also used. Mild to moderate nonspecific proliferative changes and increased vascularity of the subsynovial space were seen. Electron dense deposits and viral-like particles were not observed. Immunohistochemical staining revealed HIV p24 positive staining in cells of the synovial lining layer and in the mononuclear cells of the subsynovial space. CD4, CD8, with predominance of CD8, and B and DR cells were also present. The presence of HIV p24 antigen may be indicative of a role, yet to be defined, in the pathogenesis of HIV associated rheumatic disorders.

Acquired Immunodeficiency Syndrome

Structural and stereochemical relations between acidic macromolecules of organic matrices and crystals.

Detailed studies of the molecular organization of various mineralized tissues highlight the role played by acidic glycoproteins in controlling crystal formation. In vitro experiments show that these macromolecules are able to interact with specific faces of different crystals, influencing both nucleation and crystal growth. Significantly a common stereochemical motif can be recognized in all the interacting faces studies to date.

Animals

Psoriatic arthritis and acquired immunodeficiency syndrome.

We describe 2 patients who developed psoriatic arthritis during the course of acquired immunodeficiency syndrome. Both patients developed skin and articular involvement characteristic of psoriatic arthritis; these manifestations were refractory to conventional therapy. Our findings suggest that psoriatic arthritis should be added to the expanding spectrum of musculoskeletal manifestations of the acquired immunodeficiency syndrome.

Acquired Immunodeficiency Syndrome

Rheumatic manifestations of human immunodeficiency virus infection.

PURPOSE: The prevalence and characteristics of the rheumatic and extra-rheumatic manifestations of human immunodeficiency virus (HIV) infection were determined in a prospective manner. PATIENTS AND METHODS: One hundred one patients with HIV infection were consecutively interviewed and examined. The prevalence of autoantibodies and their association with rheumatologic symptoms were also determined. RESULTS: The musculoskeletal system was involved in 72 patients. Thirty-five patients had arthralgias, 10 had Reiter's syndrome, two had psoriatic arthritis, two had myositis, and one had vasculitis. Also found were two previously unreported syndromes. The first, occurring in 10 patients, consisted of severe intermittent pain involving less than four joints, without evidence of synovitis, of short duration (two to 24 hours), and requiring therapy (ranging from nonsteroidal antiinflammatory drugs to narcotics). The second, occurring in 12 patients, consisted of arthritis (oligoarticular in six patients, monoarticular in three patients, and polyarticular in three patients) involving the lower extremities and lasting from one week to six months. The synovial fluid of five patients (three with arthritis, one with Reiter's syndrome, and one with psoriatic arthritis) was sterile and inflammatory. CONCLUSION: Musculoskeletal complications are common in advanced stages of HIV infection. Persons in a high-risk group for HIV infection who manifest oligoarthritis with or without any other extra-articular manifestation suggestive of Reiter's syndrome or other form of spondyloarthropathy should be tested for HIV.

Acquired Immunodeficiency Syndrome

Atrophoderma (Pasini-Pierini). Findings on direct immunofluorescent, monoclonal antibody, and ultrastructural studies.

A patient with atrophoderma (Pasini-Pierini) was studied. Microscopic examination showed small collections of mononuclear cells around dermal blood vessels. Electron microscopic study demonstrated macrophages and lymphocytes around vessels and between fibers in the dermis; the epidermis, dermis, collagen, and elastic fibers appeared normal. Monoclonal antibody studies of the cells in the perivascular infiltrate demonstrated cells reacting with anti-Leu-1 (pan-T-cell antibody), anti-Leu-3a (the helper/inducer T-cell antibody), and OKM 1 antibody-reacting cells (macrophages). Direct immunofluorescent studies showed IgM and C3 staining in the small blood vessels of the papillary dermis, scattered IgM cytoids at the basement membrane, and focal fibrinogen in the mid-dermis. Mononuclear cells in the perivascular infiltrate, similar in type and percentage concentration, have been demonstrated also in patients with anetoderma, another rare atrophic cutaneous disorder. Macrophages and T lymphocytes around papillary dermal blood vessels may play a role in the pathogenesis of atrophoderma and anetoderma.

Adolescent

Dry period heat stress relief effects on prepartum progesterone, calf birth weight, and milk production.

Effects of cooling high producing dairy cows during the dry period were examined in 84 pluriparous Israeli-Holstein cows. Cooling was by a combination of wetting and forced ventilation from 0600 to 1800 h until parturition and common management afterwards for both groups. Cooling maintained diurnal increase in rectal temperature within .2 degrees C as compared with .5 degrees C in control cows in warmer months, Mean rectal temperatures at 1400 h in control cows were moderate, within 39.2 degrees C. Cooling did not affect prepartum or postpartum body condition score or mean blood progesterone during the dry period. Results suggested a possible increase in blood progesterone in later pregnancy by cooling during hot weather. Cooling increased mean 150-d milk production by 3.6 kg/d (3.1 kg FCM/d). Prepartum cooling negatively affected first lactation month yield in cows calving in early summer. Prepartum cooling might prevent adaptation to heat and impair subsequent postpartum performance. Prepartum progesterone was not related to milk yield. Calves' birth weight increased by cooling, but the effect was mostly in older cows. Birth weight was related to milk yield, independently of cooling effect, mostly in older cows. Cooling during the dry period might increase milk yield as it does during lactation. Results indicate possible benefit of cooling dry cows even under mild heat stress.

Animals

Thermal, productive, and reproductive responses of high yielding cows exposed to short-term cooling in summer.

Effect of cooling on body temperature, milk production, estrous behavior, and reproductive performance was examined in 66 estrous-synchronized, Israeli-Holstein dairy cows. Cooling was by an automated system, which actuated sprinkling (30 s) followed by forced ventilation (4.5 min) for 30-min periods. Cows were cooled 9 times/d between 0500 and 2100 h over 10 d, starting 1 d before expected estrus until d 8 post estrus. Cooling reduced typical diurnal rise of body temperature in summer heat-stressed cows by .5 to .9 degrees C, and body temperature was maintained close to normothermic temperature (38.6 degrees C). Milk production of cooled cows was 2.6 kg/d (+8%) above control at end of the cooling period. More cooled cows than noncooled exhibited standing estrous behavior; in noncooled cows, silent ovulations or anestrus were more frequent. Conception rate of cooled cows did not differ from control, suggesting need for a longer than a 10-d cooling period for improvement of fertility. The cooling system has potential to alleviate heat stress in dairy cows and to improve their thermal balance, productive, and reproductive performances.

Animals

Hyperthermia and body energy store effects on estrous behavior, conception rate, and corpus luteum function in dairy cows.

The reproductive performance of 74 Israeli Holstein dairy cows was examined during summer. Cows were fed prepartum to reach high (3.8) and low (2.6) body condition scores by 1 mo prepartum. After calving, half of each group were cooled seven times a day for 30 min by sprinkling and ventilation. Cows were inseminated starting 60 d postpartum. Daily mean body temperatures of cooled and noncooled cows were 38.6 and 39.2 degrees C, respectively, with differences between them reaching 1 degree C and more during the hot hours. Body condition affected only the time taken postpartum to the start of ovarian activity (26 d for high and 32 d for low body condition groups). Estrous behavior lasted longer in cooled (16 h) than in noncooled (11.5 h) cows of the low body condition group only. Conception rate was higher in cooled than in noncooled cows (59 vs. 17%). Pregnancy rate at 90 d postpartum was higher in cooled (44%) than in noncooled cows (14%). Progesterone concentrations were higher in inseminated nonpregnant and in noninseminated cyclic cooled cows than in noncooled cows and were similar in pregnant cows of both cooled and non-cooled groups. The present cooling method appears to have a high potential for improvement of summer fertility.

Animals