Rheumatoid arthritis and pseudo-rheumatoid arthritis in calcium pyrophosphate dihydrate crystal deposition disease

Calcium pyrophosphate dihydrate (CPPD) crystal deposition disease can lead to many clinical syndromes. One syndrome simulates rheumatoid arthritis and is thus called "pseudo-rheumatoid arthritis." Since some patients have true rheumatoid arthritis with CPPD crystal deposition disease, the clinician may have difficulty differentiating those patients from others who have the pseudo-rheumatoid syndrome. Such a diagnostic problem can be solved radiographically. Eleven patients with CPPD crystal deposition disease were studied; five had true rheumatoid arthritis and six had pseudo-rheumatoid arthritis. Because osseous erosions were not apparent in the arthropathy of uncomplicated CPPD crystal deposition disease, the detection of skeletal erosive changes indicated a true rheumatoid arthritis process.     

Lissencephaly associated with cerebellar hypoplasia and myoclonic epilepsy in a Bedouin kindred: a new syndrome?

Clinico-radiological assessment of three mentally retarded members of a large Bedouin kindred showed lissencephaly, spastic paraparesis, myoclonic epilepsy and cerebellar hypoplasia. It seems that the familial association of lissencephaly/myoclonic epilepsy/cerebellar hypoplasia represents a new entity.     

Anatomic bases of a vascularized allogenic knee joint transplantation: arterial blood supply of the human knee joint

Summary The vitality of a graft in a vascularized allogenic knee joint transplantation in humans will depend strongly on maintaining the arterial blood supply to the bone. As can be demonstrated in injected specimens on cadavers, only periostal aa. are important to ensure the blood supply of the human knee joint. The main arteries have defined nutritive areas. According to our results access to the vessels should most probably be conducted from the dorsal side of the knee. During the explantation the popliteal a. should be ligated proximally as high up as possible and distally just above its trifurcation. The articular branch of the descending genicular a. of both the donor and the recipient can be ligated because of a rich anastomotic network. The medial and lateral inferior genicular aa. of the donor should be ligated ventrally as far forward as possible. One should, above all, preserve the medial inferior genicular a. of the donor in order not to endanger the medial part of the distal segment of the graft. If an operation is to be performed according to the results of our anatomical studies, vascularized allogenic knee joint transplantations should be successful from an anatomical point of view.

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Bases anatomiques de la transplantation d'une allogreffe vascularisée de genou : vascularisation artérielle de l'articulation du genou humain

Résumé La vitalité d'une greffe lors d'une transplantation d'allogreffe vascularisée de genou dépendra principalement du maintien de la vascularisation artérielle de l'os. Comme on peut le montrer sur des spécimens cadavériques injectés, seules les artères périostées sont importantes pour assurer la vascularisation artérielle du genou humain. Les principales artères ont des territoires définis. En fonction de nos résultats, l'accès aux vaisseaux serait au mieux mené par le versant dorsal du genou. Pendant le prélèvement, l'a. poplitée devrait être sectionnée proximalement aussi haut que possible, et distalement juste au-dessus de sa trifurcation. Le rameau articulaire de l'a. descendante du genou du donneur et celui du receveur peuvent être liées en raison du riche réseau anastomotique. Les aa. géniculées inféro-médiale et inféro-latérale du donneur devraient être liées aussi loin que possible en avant. Il faudrait par dessus tout préserver l'artère géniculée inféromédiale du donneur pour éviter de léser la partie médiale du segment distal de la greffe. Si une transplantation d'un allogreffe vascularisée de genou devait être réalisée en tenant compte du résultat de nos travaux, elle devrait être un succès du point de vue anatomique.

     

Lack of breast feeding and early weaning in infants of Asian immigrants to Wolverhampton

Fifty Asian immigrant mothers who would have expected to breast feed their infants had they remained in rural Asia were studied. There was a striking reduction in the incidence and duration of breast feeding on arrival in the United Kingdom, and a fall in the age of weaning. The availability of an alternative to human milk is the most important factor reducing the incidence of breast feeding. Only 2 (4%) of the 46 infants followed prospectively were breast fed. Reasons for not breast feeding were sought and the results indicated that the majority of mothers were frightened, misinformed, or apathetic about breast feeding. If breast feeding is to be promoted, antenatal education and encouragement is essential. The advantages of human milk need to be stressed. Potentially serious mistakes occurred in preparing bottle feeds, and vitamin supplements were often inadequate. Later weaning could be encouraged by the staff of well baby clinics.     

Differential induction of gamma-glutamyl transpeptidase in primary cultures of rat and mouse hepatocytes parallels induction during hepatocarcinogenesis

In carcinogen-treated rats, gamma-glutamyl transpeptidase (GGT) is induced in preneoplastic liver lesions and liver tumors. However, in mice, GGT is rarely detected during hepatocarcinogenesis. Data in this study reveal that GGT is not induced in mouse hepatocytes when they are maintained in vitro under the same conditions that induce GGT activity in primary cultures of rat hepatocytes. GGT activity in rat hepatocytes increased 20-fold during the first 7 days in culture, but there was no induction of GGT in primary cultures of mouse hepatocytes. Comparison of intracellular glutathione levels in rat and mouse liver cells showed that the glutathione level was higher in the mouse liver cells than the rat. Blocking glutathione synthesis with buthionine sulfoximine reduced the intracellular glutathione concentration in mouse liver cells but did not trigger an induction of GGT. Analysis of the GGT mRNA in primary cultures of rat hepatocytes showed that only GGT mRNA(III) is induced. This is the same GGT mRNA species present in preneoplastic hepatic lesions and liver tumors in the rat (1-3). Therefore activation of promoter III in the GGT gene is responsible for induction of GGT in both hepatocytes in vitro and liver tumors in vivo. These data show that primary cultures of rat and mouse hepatocytes provide a model system with which to study interspecies differences in the regulation of this enzyme and to better understand the role of GGT in normal and neoplastic processes.      

Primary pulmonary tuberculosis presenting as mass lesions and simulating tumours in children

Two cases of primary pulmonary tuberculosis presenting as mass densities and simulating neoplasms in children are reported. This manifestation has not been previously reported in children. It probably represents an unusual immunological response to the mycobacterium.     

Primary infections of Epstein-Barr virus, cytomegalovirus, and human herpesvirus-6

A total of 121 infants entered a cohort serological study of primary infections with herpes-viruses. All of them had seven samples of blood available: the first sample was taken soon after birth, the other six were taken at 1, 2, 3, 6, 12, and 14 months of age. One sample of maternal blood was collected immediately after delivery. All blood samples were tested for antibodies against cytomegalovirus, Epstein-Barr virus, and human herpesvirus-6 (HHV-6). Primary cytomegalovirus infection occurred early; the cumulative infection rates were 1.7%, 8.3%, 18.3%, 25%, 52.5%, and 65% by the ages of 1, 2, 3, 6, 12, and 14 months, respectively. Epstein-Barr virus infection was not seen before 3 months of age and slowly emerged thereafter, reaching a cumulative rate of 1.7%, 11.6%, 21.5% at the ages of 6, 12, and 14 months, respectively. Primary HHV-6 infection was also a rare event in the first three months of life, but peaked between 6 and 12 months of age. No detectable risk factors were associated with primary Epstein-Barr virus or HHV-6 infection. The risk factors associated with cytomegalovirus infection included breast feeding, fewer children in household, and care by a babysitter.     

Genetic causes of chronic pancreatitis: the elucidation of genetic contributions to a disorder once thought to have none

Chymotrypsin C (CTRC) variants that diminish activity or secretion are associated with chronic pancreatitis
Jonas Rosendahl et al. (2008)
Nature Genetics 40: 78–82     

视网膜神经节细胞诱向因子单克隆抗体识别人视网膜母细胞瘤存在RGNTF

用抗大鼠视网膜神经节细胞诱向因子(Retinal ganglion neuronotrophic factor,RGNTF)单克隆抗体对临床手术切除的4例人视网膜母细胞瘤(Rb)和培养的人(Y-79)视网膜瘤细胞株进行了免疫细胞化学染色。结果显示,瘤细胞有较强的反应阳性。为进一步证实,用Y-79细胞株制备Rb细胞提取液作快速电泳,并转移至PVDF膜,用RGNTF单克隆抗体进行Western印迹反应,结果表明,RGNTF单抗仅在Rbe电泳的30kD蛋白带呈现单一的阳性反应带,其余蛋白带皆为阴性。为识别Rb瘤细胞内存在的尽GNTF与大鼠顶盖提取液中的RGNTF是否享有相同的生物效应,用辣根过氧化物酶(HRP)逆行标记的初生大鼠视网膜神经节细胞(RGC)培养在Rb提取液(Rbe)和顶盖提取液(Te)电泳后的凝胶片上48小时。结果发现HRP阳性的视网膜神经节细胞只存活在Rbe(共有18条蛋白带)与Te(共有25条蛋白带)中的30kD蛋白带上,表明Rbe中的RGNTF对RGC有同样生物效应。以上结果说明,人视网膜母细胞瘤内存在与大鼠相同的RGNTF,并提示Rb肿瘤细胞的超常生长可能与其自身产生的RGNTF有关。