Mixed connective tissue disease: a disease entity?

OBJECTIVE: To explicate whether mixed connective tissue disease (MCTD) is a distinct disease and evaluate the reliability of three different diagnostic criteria proposed by Sharp, Alarcon-Segovia and Kasukawa respectively. METHODS: Clinical follow-up of 50 MCTD patients lasted 2-8 years (80% > 5 years). HLA-A, -B as well as -DR typing was performed by complemently dependent cytotocity assay. Autoantibody profile was detected by counterimmune electrophoresis (CIE). RESULTS: Thirteen (26.0%) of the 50 MCTD patients subsequently developed other connective tissue disease (OCTD), including 7 systemic lupus erythematosis (SLE), and 6 progressive systemic scleroderma (PSS). Among 23 of the MCTD patients fulfilling Sharp's criteria, 1 (4.3%) developed PSS, but among 23 of the patients fulfilling Kasukawa's, not Sharp's, 7 (30.4%) developed OCTD and among 27 of the patients fulfilling Alarcon-Segovia's, not Sharp's, 12 (44.4%) developed OCTD. In the frequencies of DR4 and DR5, there were significant differences between patients fulfilling Sharp's (60.9%, 56.5%) and controls (24.3%, P < 0.005, RR = 4.7 and 21.4%, P < 0.005, RR = 4.6%), but there were no significant differences between the patients not fulfilling Sharp's and normal control (P > 0.05). CONCLUSIONS: MCTD is a distict rheumatic disease. Sharp's criteria is the most reliable for diagnosis of MCTD.     

Autoimmune myocarditis: a clinical entity.

In a case of myocarditis electron microscopic and immunoflourescent studies of a transmural myocardial biopsy specimen indicated an autoimmune process. Extensive inflammatory cell infiltration, immunoglobulin and complement deposition along the sarcolemma and in the interstitium, and capillary endothelial injury were found. After a short course of immunosuppressive therapy the inflammatory process was replaced by collagenous scarring and lymphocytic depletion; the blood vessels were then normal. Earlier therapy in such cases may be lifesaving.     

Angiokeratoma presenting as plantar verruca: a case study

One of the more frequent pathologic conditions that podiatric physicians are confronted with is plantar verrucae. Plantar verrucae have been studied extensively in terms of morphological features and incidence in the population at large and in patients with human immunodeficiency virus infection. Solitary angiokeratomas can be morphologically similar to plantar verrucae, presenting as hyperkeratotic pedunculated lesions. We present a unique case study of a 40-year-old man with human immunodeficiency virus with a painful solitary angiokeratoma masquerading as plantar verrucae. The lesion demonstrated clinical signs consistent with those highlighted in the literature for verrucae, namely, showing as red and black lacunae, punctuated hyperkeratotic epidermis. We propose that solitary angiokeratomas should be an important part of a podiatric physician's differential diagnosis in the lower extremity owing to the similarity of morphological features with plantar verrucae.     

Alcoholism and other drug addictions: a primary disease entity, 1991 update

Intensive work needs to continue on the genetic characteristics of the X-Y chromosome. There needs to be further clarification of the allele penetrants and the oncogenes, as well as determination of the specific chromosomes. It is now apparent that the neurotransmitters, particularly dopamine, seratonin, and norepinephrine, as related to the MFB receptor sites, are a determining factor in the presence or absence of this disease. However, the exact manner in which these neurotransmitters and receptor sites act, particularly in terms of the feed-back systems, remains obscure. New receptor sites are also being discovered and additional neurotransmitter systems, such as the second messenger system, uncovered. It would be naive at this point to attempt to delineate specific receptor sites or neurotransmitter systems as the absolute determinant of the disease. However, the basic heuristic model accepted by the Georgia Impaired Health Professionals Program remains valid. The heuristic model proposed in the past 15 years remains basically intact and valid. In this model, it is apparent that the essential disease criteria are fulfilled by alcohol and other drug addictions. Chemical dependency is a primary, psychosocial, biogenetic disease. The American culture, however, often fails to appreciate the difference between controlled abusive use and the uncontrolled addictive user. They do not understand that controlled abusive use can lead to severe physical changes, even death.(ABSTRACT TRUNCATED AT 250 WORDS)     

Chronic Caffey's disease: an uncommon entity in children

Chronic Caffey's disease in an uncommon condition in children is characterized by an acute inflammatory reaction in the periosteum along with systemic disturbances. A 30 months old boy was reported in the pediatric unit of BSMMU, Dhaka about two and half years back with the complaints of multiple painful soft tissue swelling in different parts of the body since birth and delay in growth and development. The child was found well and alert, moderately pale, febrile with hard, tender swelling of mandible on both sides. There were multiple swellings over the right arm, forearm, both thighs and bowing of the lower limbes. Investigations revealed normal serum calcium and phosphate level with mild elevation of alkaline phosphatase. Radiological findings showed periosteal new bone formation in mandible and long bones. There was diaphyseal expansion of the long bones with expansion of the ribs anteriorly. He was diagnosed as a case of chronic caffey's disease on the basis of history, clinical examination and investigation.     

Tryptophan-associated eosinophilic connective-tissue disease. A new clinical entity?

Seven patients who developed a syndrome of eosinophilia, connective-tissue disease, and cutaneous abnormalities while ingesting tryptophan were examined. Other clinical manifestations commonly seen were pulmonary symptoms, fever, lymphadenopathy, and the development of myopathy. Laboratory features included mild elevations of aldolase and lactate dehydrogenase levels, with essentially normal creatine kinase levels, erythrocyte sedimentation rates, and C-reactive protein levels. Biopsy findings included features of scleroderma, small-vessel vasculitis, fasciitis, and myopathy. Discontinuation of tryptophan administration and implementation of corticosteroid therapy were of some benefit in relieving the intense myalgias and cutaneous findings that developed. Although temporally related to tryptophan ingestion, it is unclear whether this substance, a metabolite, or a contaminant were causal. We speculate that the pathogenesis of this syndrome may relate to abnormalities in tryptophan metabolism.     

Actinomyces infection of the mastoid: a rare entity

A rare case of mastoid infection caused by actinomyces israelii is presented. This patient underwent exploratory mastoidectomy followed by long term oral pencillin. She responded well to the treatment and has been asymptomatic on follow up to date.     

Twin lithopaedions: a rare entity

Lithopaedion (stone baby) is the name given to an extrauterine pregnancy that evolves to foetal death and calcification. There are around 300 cases reported in the world medical literature to date. We report the case of a 40-year-old woman who presented with features of acute intestinal obstruction (abdominal distention, vomiting and absolute constipation) for a week. She had a past history of a missed abortion in the fifth month of gestation, eight years prior to this presentation, one which we thought to be irrelevant to the present illness. However, complementary investigations, including scout abdominal radiographs and ultrasonography of the abdomen and pelvis, were done before the operation. The abdominal radiograph showed two opaque globular masses on either side of the lower abdomen with distended small intestinal loops. Exploratory laparotomy was performed and a portion of strangulated small bowel attached to a solid globular mass behind the left ovary was removed, with a subsequent resection of the gut and end-to-end anastomosis. Upon removal of a thick membrane from the globular mass, a dead five-month-old calcified foetus was recovered. In the right iliac fossa, a similar mass was removed and another dead calcified foetus of similar age was recovered.     

HIV: evolution of a pandemic.

Although the prevalence of AIDS is still relatively low in many countries in Asia and the Pacific Rim, the rate of HIV transmission in this region continues to rise inexorably and will surpass that of sub-Saharan Africa by 1997. The challenge of mobilizing governments and communities to counter this largely invisible threat was the theme of the Third International Conference on AIDS in Asia and the Pacific, held in Chiang Mai, Thailand, in September 1995. Thailand has led the way with bold and far-reaching HIV prevention programs. Nevertheless, the long-term consequences of existing HIV infection in Thailand and elsewhere in Asia will be severe. Moreover, these repercussions will be felt globally as productivity is undermined, health care costs soar and purchasing power weakens. Supporting programs for HIV prevention and care abroad is thus an urgent matter of economic and political self-interest as well as a humanitarian imperative.