Sarcoidosis in children. Epidemiology in Danes, clinical features, diagnosis, treatment and prognosis

This paper reviews current knowledge of childhood sarcoidosis with regard to the epidemiology in Danes, clinical presentation, diagnostic procedures, treatment and prognosis. Sarcoidosis is a granulomatous disease of unknown aetiology, with multiorgan involvement. The diagnosis is confirmed by the demonstration of epitheloid cell granulomas in tissue biopsy specimens. During the period 1980-92, three cases of childhood sarcoidosis were recorded in Copenhagen County, which has a total population of 610,000. The approximate incidence of clinically recognized sarcoidosis in Danish children younger than 15 y of age was 0.22-0.27/100,000 children per year, corresponding to approximately three new cases in Denmark each year. The true incidence is unknown, since the disease is often asymptomatic and resolves without a clinical diagnosis being made. In children younger than 5 y of age, the disease is characterized by involvement of skin, eyes and joints, whereas in older children involvement of lungs, lymph nodes and eyes predominate. The mainstay of treatment consists of oral corticosteroids. The risk/benefit ratio of using long-term corticosteroids needs to be evaluated in each individual patient. Some patients may benefit from additional therapy with methotrexate. The long-term prognosis is not well established, but it seems to be poorer in children younger than 5 y. Older children appear to have as favourable a prognosis as young adults.     

Epidemiology of osteoporosis: incidence, prevalence, and prognosis

Though the exact and cumulative incidence of osteoporosis has not been examined, using the criteria that osteoporosis is defined by a decrease of bone mineral density (BMD) below 70% from young adult mean (YAM), the prevalence of osteoporosis among the Japanese women can be estimated as to be more than either 30% (by spinal BMD) or 37% (by femoral BMD) in 60's, 37% or 64% in 70's, and 42% or 90% in 80's, respectively. The total number of osteoporosis among the women with age 50 and over can be also estimated as to be either about 5 million (by spinal BMD) or 8.5 million (by forearm BMD). National survey shows that the ratio of people receiving treatment for osteoporosis is 34.5 per 1,000 among the elderly with age 65 and over. In addition, both functional transition and survival rate of discharged patients with osteoporotic hip fracture are discussed as prognosis of osteoporosis.     

Traumatic arteriovenous fistulas of the meningeal vessels. Remarks on 2 cases (author''s transl)

Serum levels of LSH, LH, estradiol, progesterone, and prolactin were measured daily for 2 months in six women after discontinuation of combination oral contraceptives. The initial LH peak occurred from 21 to 28 days after ingestion of the last tablet. Apart from variable prolongation of the follicular phase in the first postcontraceptive cycle, the patterns and levels of all hormones were indistinguishable from those found in normal ovulatory subjects. These results indicate that after a variable brief interval following discontinuation of oral contraceptive steroids, their suppressive effect on the hypothalamic-pituitary-ovarian axis disappears. This initial recovery results in completely normal endocrine function.     

Surgical treatment for Scheie's syndrome (mucopolysaccharidosis type I-S): report of two cases

Scheie's syndrome (mucopolysaccharidosis type I-S) is a rare genetic lysosomal storage disease affecting mucopolysaccharide metabolism, and is known to include cardiovascular disease. Surgical treatment was carried out in 2 patients with Scheie's syndrome. Patient 1 was a 56-year-old man with triple-vessel coronary artery disease, who successfully underwent coronary artery bypass grafting. Patient 2 was a 52-year-old man with aortic and mitral valve stenosis, who successfully underwent combined aortic and mitral valve replacement. The literature on Scheie's syndrome associated with valvular and coronary artery disease is also reviewed.     

Traumatic ostearthritis of the hip joint and its treatment (author's transl)

The author describes the various origins of osteoarthrosis following hip joint injuries. Since beginning osteoarthrosis can't be stoped, he emphasizes the surgical treatment of joint fractures including accurate reduction and stabilization of the fragments. In respect of this, intracapsular fractures of the neck of the femur always require a stabile fixation. In all cases the micro-injuries of the articular cartilage should be considered. The different possibilities of post-traumatic degenerative arthritis are discussed and the methods of operative procedures presented.     

Popliteal phlebography: principles, objectives, techniques, and results. A report on 50 cases (author's transl)

Popliteal phlebography by puncture of the popliteal vein anables direct opacification to be obtained of the deep venous trunks of the knee and the thigh. This simple technique can be used for precise evaluation of valvular incompetence. Normal and pathological findings after 50 popliteal phlebographies are described.     

The cost of the surgical treatment of cataracts at the African Institute of Tropical Ophthalmology (Bamako, Mali)

In developing countries, cataracts are a serious public health problem. There are a large number of patients, an estimated 3 millions in Africa alone, awaiting operations. However, the number of surgical interventions is small for a variety of reasons, including the cost. To try to reduce the cost of surgery, we analysed the various elements involved at the African Institute of Tropical Ophthalmology (AITO) in Bamako (Mali). The route followed by the patient, from contact with the health service through to post-operative health care was analysed in terms of individual jobs, supplies and equipment required. The unit cost of each of these elements was assessed using an adapted calculation method. The results show that the cost of the removal of the lens at the AITO is 15,200 FCFA (or $56). Installing an intra-ocular lens significantly improves the outcome for a similar cost (16,500 FCFA or $60). The relative costs of various elements were as follows: food, 32%, the suture, 12%; and equipment only 13%. Thus, to reduce the overall cost of surgery for cataracts, ambulatory surgery should be organised, with efforts to reduce the costs of suture. High quality equipment should be used, because its life-span is such that it can be amortised over a long period.     

Osteomyelitis, therapy and prognosis (author's transl)

The application of antibiotics at the treatment of osteomyelitis resulted in a rise of the permanent successful treatment rate. This is especially true for the acute hematogenous osteomyelitis, to a certain extent also for the chronic exogenous one, at which even today the antibiotical therapy is not as important as a surgical intervention. Under consideration of the generally accepted therapeutical principles the special importance of a much individualized treatment adjusted to the clinical picture and disease stage is pointed out. The advantages and disadvantages of an application of antibiotics at the osteomyelitis are mentioned and we try to explain the reasons for occasional therapy failures. A special chapter is devoted to the methods of filling and sterilization of the osteomyelitis bone hollows. Our own experiences with an operative combination process are reported.     

Pulmonary tuberculosis and primary-infection tuberculosis. Epidemiology, diagnosis, course, treatment, prevention

The herpes simplex virus type 1 (HSV-1) helicase-primase, an essential component of the viral DNA replication machinery, is a trimeric complex of the virus-coded UL5, UL8, and UL52 proteins. An assembly of the UL5 and UL52 subunits retains both enzymic activities, and the UL8 protein has been implicated in modulating these functions, facilitating efficient nuclear uptake of the complex and interacting with other viral DNA replication proteins. To further our understanding of UL8, we have constructed plasmids expressing mutant proteins, truncated at their N- or C-termini or lacking amino acids internally, under the control of the human cytomegalovirus major immediate-early promoter. Deletion of 23 amino acids from the N-terminus or 33 from the C-terminus abolished the ability of UL8 to support DNA replication in transient transfection assays. None of the UL8 mutants tested exhibited a strong dominant negative phenotype in the presence of the wild-type product, although some inhibition of replication was observed with mutants lacking 165 N-terminal or 497 C-terminal amino acids. The ability of the UL8 mutants to facilitate efficient nuclear localization of UL52 in the presence of coexpressed UL5 was examined by immunofluorescence. Selected mutants were also expressed by recombinant baculoviruses and tested for interaction with UL5 and UL52 in immunoprecipitation assays. The replicative ability of the mutants was found to correlate with their ability to localize UL52 to the nucleus, but not their interaction with UL5 and UL52. This property precluded the identification of any region of UL8 important for its presumed nuclear functions.     

Sarcoidosis in children. Clinical manifestations, epidemiology, treatment and prognosis

Sarcoidosis is a granulomatous disease of unknown aetiology, which may affect various organs. The diagnosis is obtained by the demonstration of epytheloid cell granulomas in an affected organ. The incidence of sarcoidosis in Danish children less than 15 years of age is 0.22-0.27/100.000 children/year, corresponding to approximately three new cases in Denmark each year. The disease often takes an asymptomatic course. During the period 1980-1992, three cases of paediatric sarcoidosis were observed in Copenhagen Country. All three had pulmonary involvement, and one had severe hypercalcaemia. In children less than five years of age, the disease is characterized by involvement of lungs, lymph nodes and eyes. Treatment, which is symptomatic, consists of systemic steroids. Due to the risk of growth retardation, the indication for treatment should be carefully considered and steroids administered at the lowest effective dose. Due to the lack of follow-up studies, the long term prognosis is unclarified.