Use of the index of pulmonary vascular disease for predicting long-term outcome of pulmonary arterial hypertension associated with congenital heart disease

AimsLimited data exist on risk factors for the long-term outcome of pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD-PAH). We focused on the index of pulmonary vascular disease (IPVD), an assessment system for pulmonary artery pathology specimens. The IPVD classifies pulmonary vascular lesions into four categories based on severity: (1) no intimal thickening, (2) cellular thickening of the intima, (3) fibrous thickening of the intima, and (4) destruction of the tunica media, with the overall grade expressed as an additive mean of these scores. This study aimed to investigate the relationship between IPVD and the long-term outcome of CHD-PAH.MethodsThis retrospective study examined lung pathology images of 764 patients with CHD-PAH aged <20 years whose lung specimens were submitted to the Japanese Research Institute of Pulmonary Vasculature for pulmonary pathological review between 2001 and 2020. Clinical information was collected retrospectively by each attending physician. The primary endpoint was cardiovascular death.ResultsThe 5-year, 10-year, 15-year, and 20-year cardiovascular death-free survival rates for all patients were 92.0%, 90.4%, 87.3%, and 86.1%, respectively. The group with an IPVD of ≥2.0 had significantly poorer survival than the group with an IPVD <2.0 (P = .037). The Cox proportional hazards model adjusted for the presence of congenital anomaly syndromes associated with pulmonary hypertension, and age at lung biopsy showed similar results (hazard ratio 4.46; 95% confidence interval: 1.45–13.73; P = .009).ConclusionsThe IPVD scoring system is useful for predicting the long-term outcome of CHD-PAH. For patients with an IPVD of ≥2.0, treatment strategies, including choosing palliative procedures such as pulmonary artery banding to restrict pulmonary blood flow and postponement of intracardiac repair, should be more carefully considered

A Comparison of Height Growth Curves among Girls with Different Ages of Menarche

The height growth curves of 170 Japanese girls were studied longitudinally from age 7 to 17 to see if there is a relationship with age of menarche. The height and time of menarche were obtained from school health records. The girls were divided into four groups depending on their age of menarche. The mean heights at 17 years were nearly the same among the groups. A significant relationship was found between the growth curve of height and the time of menarche. Although menarche occurs during the descent of the height velocity curve, some have the menarche soon after the time of peak velocity and still have a relatively great growth potential at the time of menarche.Discriminant analysis for the five groups of girls classified by height at 17 years of age was performed to predict adult height. When the height at the menarche, the height increment during the year prior to the menarche (PIH) and the age of menarche were used as discriminant variables; 81.2% of girls were correctly categorized. However, when PIH was eliminated,71.8% of girls were distinguished. This finding suggests that the growth after menarche is affected not only by the age of menarche but by the increment of height during the year prior to the menarche

A Case of Solitary Necrotic Nodule Treated with Laparoscopic Hepatectomy: Spontaneous Regression of Hepatocellular Carcinoma?

Solitary necrotic nodule of the liver is a rare benign lesion with a completely necrotic core and a hyalinized fibrotic capsule containing elastic fibers. The pathogenetic mechanism is still unclear. We here describe a case of SNN, whose central reticulin fibers within the nodule suggest the origin as hepatocellular carcinoma or other hepatocyte-origin tumors, treated with laparoscopic anatomical segmentectomy of the liver. A 76-year-old Japanese female, with no prior medical history and no symptom, visited our hospital with the heterogeneous hypoechoic lesion in the liver segment VI incidentally pointed out in abdominal ultrasonography. Computed tomography with contrast demonstrated a 1.1 cm sized low-density lesion with mild ring enhancement on the rim in the arterial phase. Since the possibility of malignant tumor with necrotic change could not be ruled out, she underwent laparoscopic anatomical segmentectomy of the liver. In the histological examination of the surgical specimen, the liver nodule was necrotic tissue without viable cells and signs of inflammation, which had fibrous capsule and central cystic change and showed trabecular pattern alignment of ghost cells and reticulin fibers orthogonal to the capsule. Also, the findings of chronic hepatitis were observed in the background liver

Highly sensitive diagnostic method for colorectal cancer using the ratio of free DNA fragments in serum

OBJECTIVES: The correlations of the ratio of long-/short-chain DNA fragments in blood with the existence of cancer and the clinicopathological features of colorectal cancer (CRC) were examined. The potential use of this ratio for diagnostic screening was evaluated. METHODS: DNA concentrations were amplified using Alu247 for long-chain DNA fragments and Alu115 for long- and short-chain DNA fragments. The Alu247/115 ratio was calculated for 60 patients with CRC and 24 healthy volunteers. The correlation of the Alu247/115 ratio with clinicopathological variables and the efficacy of this ratio as a tumor marker were examined. The Alu247/115 ratio cut-off value was set using a receiver operating characteristic (ROC) curve. RESULTS: The Alu247/115 ratio was significantly higher in patients with CRC than in healthy volunteers (P<0.001). The Alu247/115 ratio was also significantly higher in patients with Dukes stage A or B CRC than in healthy volunteers (P=0.034) as well as in patients with Dukes C or D CRC than in those with Dukes A or B CRC (P=0.016). Among patients with CRC, the Alu247/115 ratio was significantly higher in those with than without venous invasion (P=0.031). Using the cut-off value set from the ROC curve, the sensitivity of the Alu247/115 ratio was significantly higher than that of the carcinoembryonic antigen level (P=0.004) or the carbohydrate antigen 19-9 level (P<0.001). CONCLUSION: Our data suggest that the Alu247/115 ratio is a promising tool for highly sensitive and early detection of CRC