SED-brachydactyly and distinctive speech: report of two new cases.

We describe two unrelated patients and the mother of one of them showing clinical and radiological features as those previously described in the spondyloepiphyseal dysplasia-brachydactyly and distinctive speech (SED-BDS, also named Fantasy Island syndrome or Tattoo dysplasia) clinically characterized by short stature with acral shortness, distinctive face, mild blepharophimosis, upslanted palpebral fissures, abundant eyebrows and eyelashes, thick and abundant hair and coarse voice; and radiologically by brachymetacarpalia, brachymetatarsalia and brachyphalangia of all fingers and toes, short and broad long bones with normal morphology and small pelvis. The clinical and radiological features present in mother and son suggest a probable autosomal dominant mode of inheritance and variable expressivity.

[The prevalence of dental caries and necessary treatment in six to twelve year old schoolchildren in Goiânia, GO, Brazil, 1994]. / Prevalência de cárie e necessidades de tratamento em escolares de seis a doze anos de idade, Goiânia, GO, Brasil, 1994.

OBJECTIVE: The prevalence of dental caries and needs of treatment among schoolchildren in Goiânia-GO, middle-west of Brazil was assessed. METHODOLOGY: The study population consisted of 6-12-yr-old schoolchildren (n = 1,400) attending public schools located in the urban area of the city, where the water-supply had been fluoridated for 9 years. RESULTS: Mean dmf-t and DMF-T for the total sample were 2.19 and 2.86, respectively, showing a reduction of 57.1% in the DMF-T as compared to the regional data from the national survey carried out in 1986. At age 12 DMF-T was 4.59, which is above the acceptable level according to the FDI/WHO goal for the year 2000. Percentage of caries-free schoolchildren was very low at all ages (11% of the total sample). The most significant need was for restorative treatment in the treatment deciduous dentition at all ages and in the permanent dentition from age 9. CONCLUSION: It was concluded that the prevalence of dental caries among schoolchildren in Goiânia-GO is high, comparable to the situation in most Latin American countries and poor regions of developed countries. The results indicate the need for oral health education and preventive programs targeted at the underlying causes of the disease, in the population.

[Lymphoid myelofibrosis or hairy cell leukemia]. / La mielofibrosi linfoide o "hairy cell leukemia".

By lymphoid myelofibrosis or hairy cell leukaemia or tricholeukaemia is meant an unusual haemopathic condition known only for the past few years. It is characterized pathognomonically by the presence of lymphocyte type cells with villous extroflexions, hence the name "hairy cell". Clinically the disease presents as an involutive myelopathy associated with splenomegaly, generally without any particular lymph gland involvement. The attention of students today is concentrated on the nature of the hairy cells; while some are inclined to admit their monocyte or histiocyte derivation, others consider that they derive from B lymphocytes. Therapeutically, almost everybody agrees that splenectomy is the only valid step. A case of H.C.L., which was typical from the clinical and laboratory viewpoints is reported. It is probable that certain haemopathic pictures once classified among atypical leucoses and lymphomas, would today be more correctly classed as hairy cell leukaemia.

[Takayasu-Onishi arteritis. II. Relations of Takayasu-Onishi arteritis with other non-specific arteritides]. / Arterite di Takayasu-Onishi. Nota II. Rapporti dell'arterite di Takayasu-Onishi con altre arteriti non specifiche

Takayasu-Onishi arteritis (T.O.) is similar to Hutchison-Horton arteritis (H.H.) on histological, clinical, laboratory, and pathogenetic grounds. Both probably depend on immunitary dysreactivity, their different clinical expression being attributable to differences in the district involved and the age of the subject. Both are preceded or accompanied by rheumatism. An interesting relation can be made out between temporal arteritis and "rheumatic polymyalgia" or, more aptly, "rhizomelic polymyalgia" (Ballabio, 1975). The latter (of rheumatic origin) may accompany arteritis - Hamrin, indeed, has suggested their unification in the description "arteritic polymyalgia". It is uncertain whether vasculopathy in the course of collagen disease, rheumatic arteritis, and polyarteritis nodosa can be identified with T.O., even though a common immunological basis can be made out. The difference between T.O. and thromboangiitis obliterans, on the other hand, is quite clear at the present time.