Analysis of cerebral blood flow and intracranial hypertension in critical patients with non-hepatic hyperammonemia

Metabolic Brain Disease - Hyperammonemia in adults is generally associated with cerebral edema, decreased cerebral metabolism, and increased cerebral blood flow. The aim of this study was to...     

Two cases of dabrafenib and trametinib therapy‐failed advanced melanoma successfully controlled by nivolumab monotherapy

Although therapies for advanced melanoma have been greatly improved by the development of immune checkpoint inhibitors and BRAF/mitogen‐activated protein kinase kinase inhibitors, there are still many concerns about the administration of these novel drugs. Therefore, to combine these therapies sequentially at appropriate time points of the disease is important. In this report, we report two cases in which dabrafenib and trametinib therapy for advanced melanoma failed but were successfully controlled by nivolumab monotherapy, and investigated the sera sCD163, CCL22 and CXCL10 as biomarkers for tumor progression. Interestingly, the sera levels of sCD163, CXCL10 and CCL22, both of which are produced by activated tumor‐associated macrophages, were increased in parallel with the tumor progression in each case. Because this report presents only two cases, further data will need to be accumulated to provide more fundamental insights into the usefulness of these biomarkers for predicting disease progression in melanoma.     

Ruby laser therapy for labial lentigines in Peutz-Jeghers syndrome

Some patients with Peutz-Jeghers syndrome may be disturbed by the appearance of lentigines. Such patients require management of their lentigines as well as their gastro-intestinal polyps. We describe ruby laser therapy of labial lentigines in two children with Peutz-Jeghers syndrome. The response to treatment was excellent and no sequelae or recurrence of the lesions was noted. Conclusion Our experience suggests that ruby laser therapy is safe and a suitable approach for the treatment of labial lentigines in children with Peutz-Jeghers syndrome. Received: 5 August 1997 and revised form: 7 October 1997 / Accepted: 21 October 1997     

Review of preoperative functional evaluation for lung resection using the right ventricular hemodynamic functions.

Surgery for patients with lung cancer diminishes their lung functions, due to removal of their lung lobes. Therefore, thoracic surgeons have to consider postoperative lung function of patients. In this review, we explained recent approaches of estimation of postoperative lung function by spirometrical and also pulmonary circulatory measurement values. The most common and simple way to estimate postoperative conditions for patients who undergo lung resections is calculated by numbers of segments that are removed by surgery. However, these methods are not so accurate when the patients have chronic obstructive pulmonary disease. Another method for estimating postoperative conditions using right heart catheterization is a unilateral pulmonary arterial occlusion (UPAO) test. Applying this method, surgery related deaths have been decreased. Since, UPAO test mimics the postoperative state by occluding the pulmonary artery prior to lung surgery, it is supposed to be very accurate. Recently, a novel method to estimate postoperative right heart reserve functions was developed. Using this method, postoperative right heart failures can be anticipated prior to lung resections. In this review, we explain these kinds of methods to prevent impairment of postoperative quality of life.     

Familial neurilemmomatosis: Report of a case

We report herein a case of familial neurilemmomatosis seen in a 16-year-old girl and her 38-year-old mother. The girl presented to us with an intrathoracic vagal neurilemmoma, as well as neurilemmomas on the right fifth intercostal nerve and in the serratus anterior muscle. Two years after these tumors were resected, bilateral acoustic neuromas and multiple brain stem tumors appeared. Her mother was being treated simultaneously at another hospital for multiple neurilemmomas, including bilateral acoustic neurilemmomas, suggesting the possibility that neurilemmomatosis is an autosomal dominantly inherited disorder.     

Surgery for the patients whose total pulmonary vascular resistance was over 700 dyne during unilateral pulmonary artery occlusion test]

To determine the indication for surgery from the point of pulmonary function, we have performed the unilateral pulmonary artery occlusion test in lung cancer patients. In these patients, we gave a surgery in 13 cases whose total pulmonary vascular resistance were over 700 dyne.sec.cm-5/M2 per body surface area. The pulmonary hemodynamics before and during pulmonary artery occlusion were 549 +/- 82 and 798 +/- 78 dyne.sec.cm-5/M2 in total vascular resistance, 18.7 +/- 3.8 and 27.2 +/- 4.3 cmHg in mean pulmonary arterial pressure and 2.69 +/- 0.36 and 2.73 +/- 4.3 L/min/M2 in cardiac index respectively. In 7 cases out of 13, we performed selective pulmonary artery occlusion test. In pulmonary function test, 10 cases had a chronic obstructive diseases, 2 cases had a disturbance of diffusion and one case has a contractive disease. We gave a lobectomy in 12 cases and completion pneumonectomy in one case. Two of lobectomy cases died in the early phase: 42 and 72 days after surgery due to pulmonary complications. One of these cases needed an additional completion pneumonectomy because of bronchial fistel. Surgical complications were seen in 12 cases. These 13 cases used to be recognized that they have no indication of surgery because of low pulmonary function, however it revealed that we can give a lobectomy through a high intensive care after surgery.     

A new method for estimating filtration variables in isolated zone 1 rat lung

The filtration variables, K (filtration coefficient), Ppmv (perimicrovascular pressure) and sigma (reflection coefficient), were estimated independently in previous reports using the Starling equation or the micropuncture method. We used matrix algebra to estimate these variables simultaneously. We measured filtration rate (Q) by a gravimetric method in isolated rat lung lobes in zone 1 conditions (alveolar pressure = 20 cmH2O) at two vascular pressures, Pvasc = 15 or 18 cm H2O and perfused the lobes with plasma containing a low or a high concentration of protein. By extrapolating the log of the rate of weight gain to t = 0, we obtain the initial filtration rate before any of the pressure variables (microvascular and perimicrovascular hydrostatic pressures) in the Starling equation changed. Assuming that protein filtered into perimicrovascular space only by convection, we substituted it into the Starling equation as follows: Q = K [(Pmv -- Ppmv) -- sigma 2 (IImv)], where Pmv and IImv are microvascular and perimicrovascular plasma protein osmotic pressures. IImv was estimated by Yamada's equation (Yamada et al. 1985). For the matrix algebra, we used three values, we omitted the value for the high protein, low vascular pressure experiment. We obtained K = 26.3 [mg/(min x cmH2O x g wet weight)], Ppmv = 6.2 cmH2O and sigma = 0.46. These values agree with values from previous reports. Since these 3 filtration variables are interrelated, this new method for simultaneous measurement is more accurate than independent measurements are. The chief advantage of this method is that it does not require a separate estimate of isogravimetric pressure or a direct measurement of interstitial pressure, and all variables are obtained simultaneously.     

Polymorphic sequences of the tyrosinase gene: allele analysis on 16 OCA1 patients in Japan indicate that three polymorphic sequences in the tyrosinase gene promoter could be powerful markers for indirect gene diagnosis

Since 1989, a large number of mutations of the tyrosinase gene, which result in oculocutaneous albinism (OCA), have been reported. However, approximately 15% of patients with tyrosinase-related OCA (OCA1) heterozygously carried an uncharacterized mutation, which presumably existed outside of the ordinarily examined area of the tyrosinase gene. In such cases, polymorphic sequence(s) of the tyrosinase gene might be useful to identify the OCA1 allele. In this study, we examined four polymorphic sequences of the tyrosinase gene in 16 patients with OCA1, their relatives, and 108 normally pigmented Japanese individuals. The results showed a complex dinucleotide repeat in the promoter region at −800 to −900 of seven different lengths, and a polythymidine sequence in the 3′ end of intron 2 of three different lengths. Polymerase chain reaction-restriction fragment length polymorphism analysis of two polymorphic sequences at −301 (C/T) and −199 (C/A) in the promoter region allows us to classify the tyrosinase gene into three groups. Using these polymorphic sequences, we could identify the OCA1 allele in more than 80% of cases in which the parents' genomic DNA was available. Three polymorphic sequences in the tyrosinase gene promoter are particularly useful for this purpose. Received: July 2, 2001 / Accepted: October 5, 2001