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Background: The incidence of pulmonary hypertension (PH) associated with bronchopulmonary dysplasia (BPD) has not been investigated in regional cohorts. The aim of this study was to clarify the incidence of PH associated with BPD in all very low birthweight infants (VLBWIs) born during the study period in Aichi Prefecture, Japan., Methods: We conducted a retrospective observational cohort study of all VLBWIs born in Aichi Prefecture. The inclusion criteria were VLB, birth between 1 January 2015 and 31 December 2015, and admission to any neonatal intensive care unit in Aichi Prefecture. BPD28d and BPD36w were defined as the need for supplemental oxygen or any respiratory support at 28 days of age or 36 weeks of postmenstrual age (PMA). The primary outcome was the incidence of PH after 36 weeks' PMA (PH36w) in VLBWIs with BPD28d and BPD36w. The secondary outcomes were the clinical factors related to PH36w in BPD36w patients. Mann-Whitney U-test and Fisher's exact test were used for univariate analysis. Differences were considered statistically significant at P < 0.05. Risk ratio (RR) and 95% confidence interval (CI) were also evaluated., Results: A total of 441 patients were analyzed. A total of 217 and 131 patients met the definition of BPD28d and BPD36w, respectively. Nine patients were diagnosed with PH36w (4.2% and 6.9% of the BPD28d and BPD36w patients, respectively). The presence of oligohydramnios (RR, 2.71; 95% CI: 1.55-4.73, P = 0.014) and sepsis (RR, 3.62; 95% CI: 1.51-8.63, P = 0.025) was significant in the PH36w patients., Conclusions: The incidence of PH36w was 4.2% and 6.9% in the BPD28d and BPD36w patients, respectively. Oligohydramnios and sepsis were significantly associated with PH36w in VLBWIs., (© 2022 Japan Pediatric Society.)

An 81-year-old female was referred to our department 1 year ago due to a worsening renal function. Her manifestations met the criteria of Sjögren syndrome, suggesting renal failure likely resulting from tubulointerstitial nephritis (TIN) due to Sjögren syndrome. However, at her request, she was followed up with no further investigation or treatment. The following July, since her renal function deteriorated again, a renal biopsy was performed. Using IgM-CD138 dual staining, the renal pathology showed the infiltration of accumulated IgM-positive plasma cells within the renal insterstitium, so she was diagnosed with tubulointerstitial nephritis with IgM-positive plasma cells (IgMPC-TIN).IgMPC-TIN, proposed by Takahashi et al. in 2017, as a type of TIN, is characterized by the pathological infiltrations of IgM-positive plasma cells within the renal insterstitium and is effectively treated with corticosteroid therapy. Despite her old age, corticosteroid therapy was performed, resulting in the improvement in her renal function according to blood and urine tests and an improved pulmonary involvement, although renal dysfunction remained.Elderly patients often have multiple underlying medical conditions and take numerous medications, so differentiating renal disorders is challenging. However, a renal biopsy, even in an elderly patient, can aid in identifying the cause of renal disorders and predicting the prognosis. IgMPC-TIN is a condition in which renal function can be expected to improve if treated. It is thus important to make a diagnosis of IgMPC-TIN without overlooking and to consider proper treatment.

Infants with trisomy 18 (T18) previously had a poor prognosis; however, the intensive care of these patients has markedly diversified the prognosis. We investigated the current situation of patients with T18, clarified factors for survival discharge, and surveyed actual home healthcare. A total of 117 patients with T18 admitted to nine institutions between 2000 and 2015 were retrospectively investigated. After excluding four patients whose outcomes were unclear, we divided 113 patients into two groups-the survival discharge group (n = 52) and the death discharge group (n = 61)-and compared maternal factors, perinatal factors, neonatal factors, and therapeutic factors between the groups. In addition, home healthcare, readmission, utilization of respite care and home nursing, and cause of death among the survival group were surveyed. Fifty-two (44%) patients with T18 survived at discharge and their 1-year survival rate was 29%. The survival group had a longer gestation period, larger physique, and longer survival time, compared to the death group. Independent factors associated with survival discharge were the absence of an extremely low birthweight infant (ELBWI), the absence of esophageal atresia and patent ductus arteriosus, and cardiovascular surgery. All surviving patients required some home healthcare. The most frequent cause of death was a respiratory disorder. We recommend discussing the treatment strategy with families in the presence of neonatologists or pediatric surgeons, who can explain differences in prognosis, based on the gestation period, birthweight, severity of cardiovascular disease, and cardiovascular surgery., (© 2019 Wiley Periodicals, Inc.)

Background: Hydrops fetalis (HF) has a low survival rate, particularly in the case of preterm birth. In addition, the severity index of HF has not been fully investigated yet. The aim of this study was to clarify the prognostic factors of HF with pleural effusion., Methods: All live-born HF patients with pleural effusion, except for chromosomal abnormality or complex congenital heart disease, born from 2009 to 2013 in Aichi Prefecture in Japan were included. Prenatal, perinatal, and postnatal information was obtained from the medical records and was retrospectively analyzed., Results: Forty-one HF patients with pleural effusion were included, and 28 patients (68%) survived. On multivariate logistic stepwise analysis, gestational birth week (OR, 0.71; 95% CI: 0.52-0.96, P = 0.027) and standard deviation (SD) score of the birthweight (OR, 1.74; 95% CI: 1.01-2.99, P = 0.045) were significant factors for postnatal death. All patients with both ≥32 gestational weeks and <3.0 birthweight SD score survived., Conclusions: Combined with the gestational weeks data, birthweight SD score may be useful to estimate the prognosis of HF with pleural effusion., (© 2017 Japan Pediatric Society.)

Trisomy 13 (T13) is accompanied by severe complications, and it can be challenging to achieve long-term survival without aggressive treatment. However, recently, some patients with T13 have been receiving home care. We conducted this study to investigate factors related to home health-care transition for patients with T13.We studied 28 patients with T13 born between January 2000 and December 2014. We retrospectively compared nine home care transition patients (the home care group) and 19 patients that died during hospitalization (the discharge at death group). The median gestational age of the patients was 36.6 weeks, with a median birth weight of 2,047 g. Currently, three patients (11%) have survived, and 25 (89%) have died. The home care group exhibited a significantly longer gestational age (38.9 vs. 36.3 weeks, p = 0.039) and significantly larger occipitofrontal circumference Z score (-0.04 vs. -0.09, p = 0.019). Congenital heart defects (CHD) was more frequent in the discharge at death group, with six patients in the home care group and 18 patients in the discharge at death group (67% vs. 95%, p = 0.047), respectively. Survival time was significantly longer in the home care group than in the discharge at death group (171 vs. 19 days, p = 0.012). This study has shown that gestational age, occipitofrontal circumference Z score at birth, and the presence of CHD are helpful prognostic factors for determining treatment strategy in patients with T13., (© 2017 Wiley Periodicals, Inc.)

Ichthyosis prematurity syndrome (IPS) is a rare autosomal recessive disorder characterized by prematurity, a thick caseous scale at birth and lifelong atopic diathesis. Here, we describe the first Japanese case of IPS and report novel compound heterozygous mutations (p.C403Y and p.R510H) in fatty acid transport protein 4 (FATP4). She is the first reported patient of Asian origin, entirely distinct from the Scandinavian population, in whom the heterozygote carrier frequency is very high.

Fetal growth restriction (FGR) remains a cause of perinatal brain injury, sometimes leading to neurological and intellectual impairment. Although the mechanisms and pathophysiology of CNS injuries have not been elucidated completely, it is possible carbohydrate and energy metabolism may have an important role in the FGR brain. In this study, FGR was induced in rats by administration of synthetic thromboxane A2 (STA2). Pups were delivered by cesarean section. After killing, samples were obtained from the fetuses of both control and FGR rats for evaluation of carbohydrate and energy metabolism in brain tissue. Lactate and pyruvate levels in brain were reduced significantly in the FGR group. Glucose content in brain tissue tended to be increased in the FGR group. In contrast, glycogen content in brain tissue tended to be lower in the FGR group. However, these differences in glucose and glycogen content did not reach statistical significance. Brain high-energy reserves, including ATP, ADP, AMP, and phosphocreatine (P-Cr), were similar in the control and FGR groups. Gluconeogenesis compensated for chronic fetal hypoxia and decreased glycogen storage. Energy metabolism in the FGR brain is likely to be disrupted as a consequence of lower reserves of energy substrates.

Objective: Intrauterine growth retardation (IUGR) is an important cause of prenatal and neonatal morbidity, and neurologic abnormalities. Although several animal models of IUGR have been developed for scientific investigation, few models approximate the pathophysiology in human fetal growth failure resulting from pregnancy-induced hypertension and preeclampsia. We developed an animal model of IUGR in which fetal growth restriction was induced by administering a synthetic thromboxane A(2) analogue (STA(2)) to the mother., Methods: Timed pregnant Sprague-Dawley rats were used in this study. STA(2) was delivered into the peritoneal cavity of the pregnant female at a rate of 20 ng/h from day 13 of pregnancy. The effectiveness of this model was evaluated by monitoring the overall growth of the fetuses and neonates and measuring the weight and biochemical composition of individual organs., Results: Fetuses and neonates from the STA(2) group showed a highly significant weight reduction throughout the observation period from day 19 of gestation to postnatal day 7. Weight reduction near and at term exceeded 10% and became more pronounced during the first week after birth. Fetuses on the 20th gestational day exhibited a pattern of growth retardation characteristic of asymmetrical IUGR in which the weight reduction was prominent in the liver with relative sparing of the brain. However, the decrease in brain weight was more than 10%. The protein, DNA, and RNA contents of the liver were lower in the STA(2) group. The protein content of the forebrain and brainstem also decreased significantly in the STA(2) group compared with the control; however, the DNA content of the forebrain was higher in the STA(2) group., Conclusions: This animal model may mimic human IUGR more closely than previous models because the growth restriction is induced in a truly chronic manner.

Callus cultures of Epimedium diphyllum produced a large amount of epimedoside A in addition to a small amount of diphylloside B, ikarisoside C, epimedoside E, diglycosides of des-O-methylanhydroicaritin (8-gamma, gamma-dimethylallylkaempfero). Icariin, epimedins A-C, which are glycosides of anhydroicaritin, were also produced in the callus cultures. Contents of the flavonol glycosides in callus tissue were higher than those of mother plants, but the composition of each flavonol glycoside mixture in the callus cultures was different from that of the original plants. The time-course experiments showed that an inverse relationship existed between cell growth and flavonol glycoside production. Effects of hormonal factors on cell growth and flavonol glycoside production indicated that 2,4-dichlorophenoxyacetic acid was needed for the production of flavonol glycosides.

A rare case of metastatic renal tumor originating from a malignant mixed tumor of the submandibular gland is reported. The patient was a 45-year-old woman with hard masses palpated in the right upper arm and the right upper gluteal region. She had a history of surgical resection of a right submandibular tumor followed by radiotherapy in 1977. Biopsy findings of the gluteal mass suggested a malignant clear cell tumor. A chest X-ray, excretory urogram, CT scan, MRI and renal angiogram were highly suggestive of a right renal cell carcinoma with metastases to the right lung (S1 & S10), the left 4th and 5th ribs, the right upper arm muscle and the right upper gluteal muscle. Right nephrectomy and resection of the right upper arm and right upper gluteal tumors were performed. Microscopic examination showed that all tumors were malignant mixed tumors. When compared to the previous surgical specimen, the tumors were believed to be identical. The tumors of the lung and ribs subsequently resected were malignant mixed tumors. The patient is well with no objective or subjective evidence of recurrent neoplastic disease 7 months post-operatively. To our knowledge only one report of metastatic renal tumor originating from a malignant mixed tumor of the salivary gland has been published in Japan. But metastatic renal tumor originating from the same tumor of the submandibular gland has not been reported previously in Japan.

Absorption of tetrahydropyranyl adriamycin (THP) administered immediately after transurethral resection of bladder carcinoma (TUR-Bt) has not been reported. In this study, we have examined the absorption of THP and the systemic toxicity in the early post-TUR period. Of 21 patients with bladder carcinoma, 10 had a solitary tumor and 11 multiple tumors. Twenty mg THP in 40 ml of sterile water was intravesically administered on days 1, 3, 5, 7, 14 and 28, and then every 4th week. The THP solution was retained for 2 hours. The blood THP concentration was measured 30 minutes and 2 hours after the intravesical administration on days 1, 7 and 28. No systemic side effects were observed. Thirteen of the 38 (34%) samples contained a detectable level (more than 1 ng/ml) of THP on the post-TUR-Bt on day 1, 8, of 42 (19%) on day 7, and 3 of 18 (17%) on day 28. Altogether, 24 of the 98 (24%) samples contained more than 1 ng/ml THP. The highest blood THP level was 23 ng/ml on day 1. The differences between frequency of detection of blood THP in the samples at 30 minutes and 2 hours were not statistically significant. The difference between average concentration of blood THP of patients with solitary and multiple tumors also was not significant. These results indicate that intravesical THP administration starting within 24 hours after TUR-Bt can not result in significant systemic absorption of THP, and the systemic toxicity can be avoided.

To obtain the maximum prophylactic effect of intravesical chemotherapy on the bladder tumor recurrence treatment should be started early. Since 1983, we have been initiating prophylactic instillation of doxorubicin hydrochloride (DXR) on the first post-transurethral resection (TUR) day. This study was conducted to define serum uptake of DXR and systemic toxicity in the early post-TUR period. Fifteen TURs were carried out on 14 patients with superficial bladder carcinoma. DXR (30 mg) in normal saline (30 ml) was intravesically administered 1, 3, 5, 7 and 14 days after TUR, and every 4 weeks thereafter. DXR solution was kept in the bladder for 2 hours. The serum DXR concentration was measured 30 minutes and 2 hours after the instillation through 1 to 5 days after TUR. Intolerable vesical irritability was seen in 4 of 60 instillations. No systemic side effects, however, were observed. Three of 24 samples contained a detectable level (10 ng/ml) of DXR on the post-TUR day 1, 6 of 22 samples on the post-TUR day 3, and 6 of 14 samples on the post-TUR day 5. Overall, 15 of 60 samples contained more than 10 ng/ml DXR. The highest serum DXR level was 47 ng/ml at the post-TUR day 1. Frequency of detection and average levels of serum DXR in 30-minute and 2-hour samples were not significantly different. Average concentrations in patients with multiple or diffuse tumor and solitary tumor were also not significantly different. These results indicate that intravesical instillation starting within 24 hours after TUR does not produce significant serum uptake of DXR, and systemic toxicity can thereby be avoided.

A 61-year-old female presented with gross hematuria and left flank pain. The arteriogram and CT scan revealed a large left renal tumor and multiple small right renal metastatic tumors. A chest X-ray and Ga scintigram showed no other metastatic lesions. A left radical nephrectomy and a right renal bench surgery were performed at the same time. The right multiple tumors were enucleated and the right kidney was autotransplanted into the right iliac fossa. The postoperative course was uneventful and the patient was free of tumor with good renal function. Renal preservation surgery was considered more beneficial for this patient than bilateral nephrectomy followed by hemodialysis.

One invasive and 4 superficial bladder cancers were treated by local injection of absolute ethanol through an endoscope. With the patient placed in the lithotomy position, an endoscope was introduced after mucosal anesthesia with xylocaine jelly. According to the usual manner of retrograde catheterization, a 23G syringe needle attached to a 5F ureteral catheter was advanced into the base of the tumor and through the needle absolute ethanol was injected. This treatment is characterized by endoscopic delivery of ethanol as with sclerotherapy for esophageal varices to obtain a potent necrotizing effect as achieved by transcatheter embolization of ethanol for the treatment of renal cell carcinoma. As a rule, this technique does not require any anesthesia other than mucosal anesthesia with xylocaine jelly and can be done repeatedly and very easily, thus having a high cost effectiveness. This treatment serves as a good palliative therapy for invasive bladder cancers, because of its rapid achievement of hemostasis and reduction of the tumor masses. Furthermore, this treatment is just as effective as standard transurethral resections to eradicate superficial bladder cancer, even though the treatment may have to be repeated.

Two patients with primary gastric lymphoma, diffuse lymphoma, a plasmacytoid variant of small cell type, with numerous intranuclear inclusions, are reported. In both cases, biopsy and surgical specimens showed diffuse proliferation of lymphoid cells having plasmacytoid features at different steps of maturation in lamina propria and submucosal layer. The cytoplasm of the lymphoma cells was stained with anti-IgM, anti-kappa chain, and anti-J chain antibodies by avidin-biotin peroxidase complex technique. A number of intranuclear inclusions in the lymphoma cells were diastase-resistant PAS positive, and also stained with anti-IgM, anti-kappa chain, and anti-J chain antibodies, suggesting a monoclonal situation. In an ultrastructural study of the first case, the inclusions were round and composed of amorphous materials surrounded by a single membrane. In many lymphoma cells, the perinuclear cisternal space was dilated and filled with materials similar to those within both inclusions and rough endoplasmic reticulum. A direct communication between the perinuclear space and the inclusion was demonstrated in some cells, suggesting the perinuclear cisternal origin of these inclusions.

Chemical analysis of 238U, 232Th and 40K in the dentine as well as enamel of elephant tooth fossil has been carried out in order to estimate the internal absorbed dose rate of the specimens, which was estimated to be (39 +/- 4) mrad/y on the assumption of early uptake model of radionuclides. The external radiation dose rate in the soil including the contribution from cosmic rays was also estimated to be (175 +/- 18) mrad/y with the help of gamma-ray spectroscopic techniques of the soil samples in which the specimens were buried. The 60Co gamma-ray equivalent accumulated dose of (2 +/- 0.2) x 10(4) rad for the tooth enamel gave "ESR age" of (9 +/- 2) x 10(4) y, which falls in the geologically estimated range between 3 x 10(4) and 30 x 10(4) y before the present.