Hypersensitivity pneumonitis: evaluation with CT

Thirteen chest radiographs and computed tomographic (CT) scans obtained from 11 patients with hypersensitivity pneumonitis were reviewed. The CT findings were correlated with open lung biopsy findings in seven patients. The two patients with acute hypersensitivity pneumonitis showed air-space opacification on CT scans. An open lung biopsy, done in one of these patients, demonstrated noncaseating granulomas and filling of the air spaces with macrophages. The nine patients with subacute hypersensitivity pneumonitis showed small, rounded opacities and patchy air-space opacification on CT scans. These findings reflected the histologic findings, which consisted of interstitial pneumonitis, cellular bronchiolitis, and small, noncaseating granulomas. The six patients with symptoms for 12 months or longer also showed irregular linear opacities on CT scans, corresponding to areas of fibrosis. CT scans were superior to radiographs in helping to assess the type and extent of abnormalities, and high-resolution CT scans were superior to conventional CT scans.     

沙赛普肽注射液的细菌内毒素检测

目的：用鲎试剂检测沙赛普肽注射液中的细菌内毒素。方法：采用鲎试法（LT）与家兔法（RT）对沙赛普肽注射液进行细菌内毒素检测。结果：两种方法检查结果一致,用直接稀释法排除细菌内毒素的干扰。结论：以鲎试法用于控制沙赛普注射液的热原方法可行。     

Infantile hypertrophic pyloric stenosis: An association in twins?

BACKGROUND

The etiology of infantile hypertrophic pyloric stenosis (IHPS) remains a mystery. Some suspected risk factors include birth rank, maternal age, sex, family history and monozygosity in twins. Various theories attempt to explain the etiology of IHPS. Scientific research suggests that enteric neuronal damage and nitric oxide synthase dysfunction may be implicated, but the consensus is that environmental modification must exist to account for the variability in its occurrence.

METHOD

Four cases of concordant occurrences of IHPS in twins were examined to determine the history and outcome of IHPS development in twins. Three sets were dizygotic and one was monozygotic. Of the eight infants, three were female, including the one monozygotic pair. In all four cases, a time lag existed between the development of symptomatic onset of IHPS in twin A and twin B. In one set, sonographic confirmation, performed because of IHPS diagnosis in the twin sibling, occurred concurrently with onset of vomiting, leading to early surgery before fluid and electrolyte imbalances developed.

CONCLUSIONS

Despite the lack of agreement as to whether the cause of IHPS is genetic, environmental or both, the high concordance rate seen in twins is indisputable. Thus, the empirical evidence provides credence to consider examining the asymptomatic co-twin when one of the twins presents with IHPS.     

Human erythrocyte protein 4.2 deficiency associated with hemolytic anemia and a homozygous 40glutamic acid-->lysine substitution in the cytoplasmic domain of band 3 (band 3Montefiore)

Red blood cell (RBC) protein 4.2 deficiency is often associated with a moderate nonimmune hemolytic anemia, splenomegaly, and osmotically fragile RBCs resembling, but not identical to, hereditary spherocytosis (HS). In the Japanese type of protein 4.2 deficiency (protein 4.2Nippon), the anemia is associated with a point mutation in the protein 4.2 cDNA. In this report, we describe a patient with moderate and apparently episodic nonimmune hemolytic anemia with splenomegaly, spherocytosis, osmotically fragile RBCs, reduced whole cell deformability, and abnormally dense cells. Sodium dodecyl sulfate- polyacrylamide gel electrophoresis analysis of the proposita's RBC membrane proteins showed an 88% deficiency of protein 4.2 and a 30% deficiency of glyceraldehyde-3-phosphate dehydrogenase (band 6). Structural and molecular analyses of the proposita's protein 4.2 were normal. In contrast, limited tryptic digestion of the proposita's band 3 showed a homozygous abnormality in the cytoplasmic domain. Analysis of the pedigree disclosed six members who were heterozygotes for the band 3 structural abnormality and one member who was a normal homozygote. Direct sequence analysis of the abnormal band 3 tryptic peptide suggested that the structural abnormality resided at or near residue 40. Sequence analysis of the proposita's band 3 cDNA showed a 232G-->A mutation resulting in a 40glutamic acid-->lysine substitution (band 3Montefiore). Allele-specific oligonucleotide hybridization was used to probe for the mutation in the pedigree, showing that the proposita was homozygous, and the pedigree members who were heterozygous for the band 3 structural abnormality were also heterozygous for the band 3Montefiore mutation. The band 3Montefiore mutation was absent in 26 chromosomes from race-matched controls and in one pedigree member who did not express the band 3 structural abnormality. In coincidence with splenectomy, the proposita's anemia was largely corrected along with the disappearance of most spherocytes and considerable improvements of RBC osmotic fragility, whole cell deformability, and cell density. We conclude that this hereditary hemolytic anemia is associated with the homozygous state for band 3Montefiore (40glutamic acid-->lysine) and a decreased RBC membrane content of protein 4.2. We speculate that band 3 structural abnormalities can result in defective interactions with protein 4.2 and band 6, and in particular, that the region of band 3 containing 40glutamic acid is involved directly or indirectly in interactions with these proteins.     

Complicated gastroschisis and maternal smoking: a causal association?

The incidence of gastroschisis appears to be rising in developed nations, with epidemiological studies indicating association with young maternal age and smoking. Is there an association between maternal smoking and the development of complicated gastroschisis? A retrospective population-based review of all cases of gastroschisis over 11 years was conducted in three Canadian provinces. Complicated cases were defined as those with an associated intestinal atresia or other vascular compromise of the bowel, those requiring a prolonged time to full enteral feeding (>42 days). Univariate and multivariate regression analyses were conducted. Fifty-four cases of gastroschisis were treated. Seventeen patients had complicated gastroschisis (CG). 47.1% of infants were born to smoking mothers; of those 56.25% were in the CG group. On univariate analysis statistically significant associations with complicated cases were young maternal age (<19 years) (OR = 6.0, CI: 1.3, 28.1), low maternal weight gain (<12 kg) (OR = 4.6, CI: 2.5, 8.5), low birth weight (<2,400 g), (OR = 7.7, CI: 2.0, 28.9), and gestational age under 36 weeks (OR = 11.0, CI: 2.6, 46.2). Seven of 24 cases involving maternal smoking developed atresias or vascular intestinal compromise versus 4 of 27 cases with no maternal smoking, with an odds ratio of 2.1 (CI: 0.60, 7.2). On multivariate analysis, atresias and vascular compromise remained significantly associated with young maternal age, low gestational age and low 1-min Apgar score. Although a positive trend is seen, no statistically significant relationship between maternal smoking and the development of complicated gastroschisis was found. Further clinical and epidemiologic studies are needed to validate our findings.     

Rocky Mountain spotted fever

Rocky Mountain spotted fever (RMSF) is an unusual but important dermatological condition to identify without hesitation. The classic triad of headache, fever, and a rash that begins on the extremities and travels proximally to involve the trunk is found in a majority of patients. The cutaneous centripetal pattern is a result of cell to cell migration by the causative organism Rickettsia rickettsii. Such individuals should receive prompt antimicrobial therapy and supportive care to avoid serious and potentially fatal complications.