Lethal neonatal Menkes' disease with severe vasculopathy and fractures

A male neonate presented with an acute onset of severe intra-abdominal bleeding, haemorrhagic shock and multiple fractures leading to death on d 27. Menkes' disease was diagnosed at autopsy and confirmed by copper accumulation studies on cultured fibroblasts. Such an early onset of fatal complications in this condition has not been previously reported. New insights into the pathogenesis of Menkes' disease provided by DNA mutation analysis and difficulties in neonatal diagnosis are discussed. Menkes' disease should be considered in male infants with pathological fractures and other signs of connective tissue disease, even in the neonatal period.     

Dummies and the sudden infant death syndrome

The association between dummy use and sudden infant death syndrome (SIDS) was investigated in 485 deaths due to SIDS in the postneonatal age group and compared with 1800 control infants. Parental interviews were completed in 87% of subjects. The prevalence of dummy use in New Zealand is low and varies within New Zealand. Dummy use in the two week period before death was less in cases of SIDS than in the last two weeks for controls (odds ratio (OR) 0.76, 95% confidence interval (CI) 0.57 to 1.02). Use of a dummy in the last sleep for cases of SIDS or in the nominated sleep for controls was significantly less in cases than controls (OR 0.44, 95% CI 0.26 to 0.73). The OR changed very little after controlling for a wide range of potential confounders. It is concluded that dummy use may protect against SIDS, but this observation needs to be repeated before dummies can be recommended for this purpose. If dummy sucking is protective then it is one of several factors that may explain the higher mortality from SIDS in New Zealand than in other countries, and may also explain in part the regional variation within New Zealand.     

CAESAREAN SECTION — CHANGING TRENDS DURING LAST TWO DECADES

Changing trends of caesarean section during last twenty years have been studied. There seems to be a gradual increase in the incidence of caesarean section over the years with a simultaneous decline in the perinatal mortality rate. The incidence of caesarean section has slowly been increasing for post-caesarean pregnancy, breech presentation and foetal distress. This opens up a debate as to what extent this increase is justifiable. The trend needs a close observation.KEY WORDS: Cesarean section, Trends     

Cutaneous T-cell lymphoma treated with electron beam irradiation in Indian patients

BACKGROUND: Cutaneous T-cell lymphoma (CTCL) is a rare occurrence in India. Total skin electron irradiation (TSEI) is a well-accepted therapeutic modality for the treatment of CTCL throughout the world. The aim of this study was to retrospectively analyze the treatment outcome of TSEI in Indian patients with CTCL and to determine the different parameters affecting the disease-free survival in these patients. METHODS: Fourteen male patients between 27 and 82 years of age with CTCL (duration of disease, 4 months to 2 years) were treated with TSEI between 1985 and 1998. Seven patients had early stage disease, while the other seven had advanced disease. Two patients had lymph node involvement at the time of presentation. The TSEI was performed according to the Stanford technique delivering a total dose in the range 8-36 Gy. RESULTS: Of the 14 patients, 10 showed complete remission following TSEI. The total follow-up period was 4-110 months (median, 52 months). Five patients were disease free at the end of 5 years. Two patients died due to rapid progression of the disease, while the cutaneous lesions relapsed in three patients after 2-27 months and one patient developed visceral metastasis. CONCLUSIONS: TSEI was an effective therapeutic modality for the treatment of CTCL in this group of patients, both as a curative and palliative measure, although the long-term prognosis is poor.     

Small unilateral jaw gap variations: equilibrium changes, co-contractions and joint forces

Summary  After complex prosthetic reconstructions, small differences in vertical distances between the left and right side of the jaw may occur during jaw closing, nevertheless providing bilateral tooth contacts in intercuspation by small deformations of the mandible. Their effects on the co-contraction of the masticatory muscles, the temporomandibular joint reaction forces, and the point of application of the resultant bite force vector in the maxillary occlusion plane – the so-called reduction point – have not been investigated, thus far simultaneously in one sample. The main goal of this study was to investigate variations of these measures in an experimental intercuspation simulated by one anterior and two posterior force transmission points.