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Biomedical subjects

A M Nelson

Publications and source records attributed to A M Nelson.

At least 55 records · Page 3Linked to original sources

Autosomal dominant granulomatous arthritis, uveitis, skin rash, and synovial cysts.

In 1985, Blau reported a family with 11 members in four generations affected by granulomatous arthritis, iritis, skin rash, and periarticular synovial cysts. We report a second family with these abnormalities, thereby confirming this syndrome as a distinct familial entity with transmission compatible with autosomal dominant inheritance. Affected members in our family included a mother and two daughters. Disease onset was at 10 months to 8 years of age. Each had uveitis, symmetric polyarthritis, and synovial cysts overlying the ankle and wrist joints. In addition, both daughters had an intermittent generalized erythematous papular rash that on biopsy revealed noncaseating granulomatous infiltration. All three patients improved during alternate-day steroid therapy. Recognition of this disorder as distinct from other, more common causes of arthritis is important because of the apparent autosomal dominant transmission and because of the excellent responses to low-dose steroid therapy.

Adult↗

Kawasaki syndrome.

Kawasaki syndrome is an acute, self-limited illness of young children which is characterized by prolonged fever, mucositis, skin changes, and cervical lymphadenopathy. Most investigators favor a microbial agent or agents as the trigger of KS followed by an immune-mediated vasculitis with a predilection for the coronary arteries. Serious complications include coronary artery aneurysms, aneurysmal thrombosis, and death. Aspirin has been the conventional treatment for KS, but it has not been shown to alter the basic pathology. Recently intact intravenous gamma globulin has been shown to reduce the frequency of coronary artery abnormalities. Definitive therapy of KS, however, awaits the discovery of its cause and pathogenesis.

Child↗

Perinatal transmission of the human immunodeficiency virus type 1 to infants of seropositive women in Zaire.

To examine perinatal transmission of the human immunodeficiency virus type 1 (HIV-1) in Zaire, we screened 8108 women who gave birth at one of two Kinshasa hospitals that serve populations of markedly different socioeconomic status. For up to one year, we followed the 475 infants of the 466 seropositive women (5.8 percent of those screened) and the 616 infants of 606 seronegative women matched for age, parity, and hospital. On the basis of clinical criteria, 85 of the seropositive women (18 percent) had the acquired immunodeficiency syndrome (AIDS). The infants of seropositive mothers, as compared with those of seronegative mothers, were more frequently premature, had lower birth weights, and had a higher death rate in the first 28 days (6.2 vs. 1.2 percent; P less than 0.0001). The patterns were similar at the two hospitals. Twenty-one percent of the cultures for HIV-1 of 92 randomly selected cord-blood samples from infants of seropositive women were positive. T4-cell counts were performed in 37 seropositive women, and cord blood from their infants was cultured. The cultures were positive in the infants of 6 of the 18 women with antepartum T4 counts of 400 or fewer cells per cubic millimeter, as compared with none of the infants of the 19 women with more than 400 T4 cells per cubic millimeter (P = 0.02). One year later, 21 percent of the infants of the seropositive mothers had died as compared with 3.8 percent of the control infants (P less than 0.001), and 7.9 percent of their surviving infants had AIDS. We conclude that the mortality rates among children of seropositive mothers are high regardless of socioeconomic status, and that perinatal transmission of HIV-1 has a major adverse effect on infant survival in Kinshasa.

AIDS Serodiagnosis↗

Joint pain in children. When is it serious?

Diagnosis of the cause of joint pain in children depends on the physician's ability to distinguish benign from more serious joint conditions. Benign conditions include trauma, overuse syndromes, hypermobility syndrome, chondromalacia patellae, benign recurrent limb pains, and psychogenic rheumatism. Conditions that require further evaluation and ongoing therapy include Lyme disease, rheumatic fever, juvenile rheumatoid arthritis, neoplastic disease, various orthopedic conditions, infection, seronegative spondyloarthropathies, and the rarer connective tissue diseases. If diagnosed early and treated appropriately, most joint problems of childhood have a very good prognosis.

Adolescent↗

Persistent diarrhoea in Zairian AIDS patients: an endoscopic and histological study.

To determine the aetiology of persistent diarrhoea in African patients with acquired immunodeficiency syndrome (AIDS), 42 patients with human immunodeficiency virus (HIV) and persistent diarrhoea were enrolled in a microbiological, endoscopic, and histological study. Cryptosporidium was the intestinal parasite most often identified (30%); Isospora belli was found in 12% of the patients. Histological examination of the duodenal mucosa showed a non-specific inflammatory reaction in a significantly higher number of HIV-seropositive patients (82%) than HIV-seronegative controls without diarrhoea (52%) (p = 0.02). Lymphocytes were more likely to be found in inflammatory reactions in HIV-seropositive patients than in controls (p less than 0.0001). Pathogens were observed in histological sections of the duodenum of HIV-seropositive patients only (p = 0.002) and included cryptosporidia (four patients) Isospora belli (one), Strongyloides stercoralis (one), and Cryptococcus neoformans (one). On histological examination the rectal mucosa of HIV-seropositive patients and controls was similar, except eosinophils were more likely to be present in inflammatory reaction in HIV-seropositive patients (p = 0.05) and enteric pathogens were observed only in HIV-seropositive patients (cytomegalovirus inclusion bodies (one) and Schistosoma mansoni (two). The aetiology of persistent diarrhoea in most African AIDS patients remains unclear.

Democratic Republic of the Congo↗

Liesegang rings in tissue. How to distinguish Liesegang rings from the giant kidney worm, Dioctophyma renale.

Liesegang rings (LRs) are periodic precipitation zones from supersaturated solutions in colloidal systems. They are formed by a process that involves an interplay of diffusion, nucleation, flocculation or precipitation, and supersaturation. Examples include LRs of calcium carbonate in oölitic limestone (in nature), LRs of silver chromate in gelatin (in vitro), and LRs of glycoprotein in pulmonary corpora amylacea (in vivo). Here we describe LRs in lesions from 29 patients--mostly lesions of the kidney, synovium, conjunctiva, and eyelid. The LRs formed in cysts, or in fibrotic, inflamed, or necrotic tissue. The LRs in this study varied greatly in shape and size, measuring 7-800 microns. Special stains and energy-dispersive radiographic analysis or scanning electron microscopy revealed that some LRs contained calcium, iron (hemosiderin), silicon, and sulfur. Some pathologists have mistaken LRs for eggs, larvae, or adults of the giant kidney worm, Dioctophyma renale. D. renale is a large blood-red nematode that infects a variety of fish-eating mammals, especially mink. Fourteen documented infections of humans have been recorded, usually with adult worms expelled from the urethra. The adult worms are probably the largest helminth to parasitize humans. Eggs of D. renale are constant in size (60-80 microns X 39-47 microns), contain an embryo, and have characteristic sculpturing of the shell. Liesegang rings should not be mistaken for eggs, larvae, or adults of D. renale, or for any other helminth.

Chemical Precipitation↗

Takayasu's arteritis and juvenile rheumatoid arthritis.

We describe a patient with the onset of polyarticular juvenile rheumatoid arthritis at age 7; she developed Takayasu's arteritis 12 years later. Her arthritis had been inactive until 15 months before the diagnosis of Takayasu's arteritis. The association of arthritis and Takayasu's arteritis is discussed.

Aortic Arch Syndromes↗

Fractures after rheumatoid arthritis. A population-based study.

In a population-based study, the incidence of osteoporotic fractures in patients who have been diagnosed as having rheumatoid arthritis was investigated. This incidence was found to be increased, though not dramatically so: the relative risk for hip fracture, for example, was 1.5. Univariate analyses generally indicated increased risk associated with increasing age, earlier age at diagnosis of rheumatoid arthritis, disability, impaired ambulation, steroid use, and thinness, and decreased risk associated with obesity and estrogen use. In multivariate analyses, only aging, impaired ambulation, and thinness were identified as independent risk factors.

Adult↗

Thyroid T4 5'-deiodinase activity in normal and abnormal human thyroid glands.

To investigate the activity of the thyroid gland to convert T4 to T3, we measured the activity of thyroid T4 5'-deiodinase in the following human thyroid glands: 9 normal glands, 5 Hashimoto's thyroiditis, 13 follicular adenomas, 11 methimazole (MMI)-treated Graves' disease (GD), 11 propranolol iodide-treated GD, and 8 propylthiouracil (PTU)-treated GD. The enzyme activity was determined by the ability of 100,000 X g pellet of the thyroid homogenate to convert T4 to T3 in vitro. Normal thyroids showed the enzyme activity of 1.59 +/- 0.18 (mean +/- SEM) pmol T3/mg protein/min. Euthyroid Hashimoto's thyroiditis displayed the enzyme activity of 1.01 +/- 0.15 pmol T3/mg protein/min, which was similar to the normal thyroid enzyme activity. The hypothyroid gland of Hashimoto's thyroiditis showed the enzyme activity of 1.8 pmol T3/mg protein/min. Follicular adenomas showed a wide range of enzyme activity with the mean level of 3.24 +/- 0.82 pmol T3/mg protein/min that did not differ significantly from that of the normal thyroids. Interestingly, one adenoma, despite TSH suppression that ordinarily decreases enzyme activity, showed the greatest activity of 11.0 pmol T3/mg protein/min. Graves' thyroids following treatment with MMI, PTU, and propranolol-iodide showed enzyme activities of 4.61 +/- 0.53, 3.95 +/- 0.43, and 3.51 +/- 0.46 pmol T3/mg protein/min, respectively; all these values were greater than that of the normal thyroids (P less than 0.01), but did not differ significantly when compared with each other. In summary, thyroid glands with Hashimoto's thyroiditis had activities of T4 to T3 conversion similar to the normal thyroid glands.(ABSTRACT TRUNCATED AT 250 WORDS)

Adenoma↗

Cystic duct fistula: a complication of cholecystectomy.

Two patients with postcholecystectomy cystic duct fistulas are described. Although still a most uncommon diagnosis, newer imaging procedures have facilitated recognition of this problem. Cystic duct fistulas may contribute to the subhepatic collection found in many patients postcholecystectomy.

Adult↗