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SMARCA4-deficient thoracic sarcomatoid tumor is a rare malignancy indicating some characteristics of a smoking-related disease. The purpose of this report is to describe a case of aggressive thoracic tumor with loss of immunochemical SMARCA4 expression and detail the results of our treatment regimen. The patient was a 58-year-old male and clinicopathologically diagnosed with a SMARCA4-deficient thoracic sarcomatoid tumor. Pembrolizumab plus carboplatin and pemetrexed resulted in significant response. This combination therapy showed potential for first-line systemic treatment of SMARCA4-deficient thoracic sarcomatoid tumors.

Processing ultra-fast optical signals without optical/electronic conversion is in demand and time-to-space conversion has been proposed as an effective solution. We have designed and fabricated an arrayed-waveguide grating (AWG) based optical spectrum control circuit (OSCC) using silica planar lightwave circuit (PLC) technology. This device is composed of an AWG, tunable phase shifters and a mirror. The principle of signal processing is to spatially decompose the signal’s frequency components by using the AWG. Then, the phase of each frequency component is controlled by the tunable phase shifters. Finally, the light is reflected back to the AWG by the mirror and synthesized. Amplitude of each frequency component can be controlled by distributing the power to high diffraction order light. The spectral controlling range of the OSCC is 100 GHz and its resolution is 1.67 GHz. This paper describes equipping the OSCC with optical coded division multiplex (OCDM) encoder/decoder functionality. The encoding principle is to apply certain phase patterns to the signal’s frequency components and intentionally disperse the signal. The decoding principle is also to apply certain phase patterns to the frequency components at the receiving side. If the applied phase pattern compensates the intentional dispersion, the waveform is regenerated, but if the pattern is not appropriate, the waveform remains dispersed. We also propose an arbitrary filter function by exploiting the OSCC’s amplitude and phase control attributes. For example, a filtered optical signal transmitted through multiple optical nodes that use the wavelength multiplexer/demultiplexer can be equalized.

A 50-year-old man presented with hypopituitarism and a pituitary lesion on magnetic resonance imaging scan. He was diagnosed as having lymphocytic hypophysitis, and replacement therapy with hydrocortisone and thyroxine was started. He regained normal pituitary function after 10 months. Reports of spontaneous recovery from lymphocytic hypophysitis in men are rare. While the natural history of lymphocytic hypophysitis remains elusive and its management is not well established, our report shows that spontaneous resolution may occur with steroid supplementation even in men.

Older boys benefit from higher initial prednisolone therapy for nephrotic syndrome. Background A long course of the initial prednisolone therapy has been shown to be more effective than standard-course therapy in reducing relapse rates in children with idiopathic nephrotic syndrome, but it is commonly accompanied by corticosteroid toxicities. There has been no study on prednisolone dosage for the effective treatment of nephrotic syndrome. Methods Sixty-eight children (42 boys and 26 girls) with an initial attack of nephrotic syndrome were randomly allocated into two different long-course treatment groups. Patients in Group 1 received a daily prednisolone dose of 60 mg/m 2 for six weeks, followed by an alternate-day dose of 40 mg/m 2 for six weeks. Patients in Group 2 had a daily dose of 40 mg/m 2 instead of 60 mg/m 2 . Results Four children in each group did not respond within six weeks. Group 1 was associated with a significantly earlier response but more frequent corticosteroid toxicities than Group 2. Boys in Group 1 had a higher rate of sustained remission than boys in Group 2 ( P = 0.0073), especially boys four years old or more ( P = 0.0027), but girls did not show a significant difference ( P = 0.863 ). Boys four years old or more in Group 1 had a course of frequent relapsing less often than those in Group 2 (2 of 13 vs. 6 of 8, P = 0.0075). Conclusion These findings indicate that efficient prednisolone doses may vary between sexes and ages, and that a higher initial prednisolone therapy may be of greater benefit to older boys.

A 76-year-old man with hypoglycemic coma was diagnosed as malignant insulinoma with multiple hepatic metastases. Embolization was done for two-thirds of the hepatic mass and it rapidly lowered the serum immunoreactive insulin. He was discharged without medication and has been free from hypoglycemia. After the embolization, the serum creatine kinase (CK) level increased transiently although there was no evidence of myocardial infarction. On electrophoresis, the CK activity showed an abnormal peak, suggesting mitochondrial CK. CK release after embolization has been reported in only a few cases with endocrine tumors, which might indicate some relationship between active energy metabolism and mitochondrial CK.

Structures are going to deteriorate from the beginning of its usage. The deteriorate function is modeled by two types in this paper and applied to the maintenance and repair management. The life cycle cost (LCC) of structures including maintenance and repair cost is minimized based on principle of minimum total cost theory. The spending cost in the life of structures has worth at each time. It is transferred to present worth to estimate the LCC. LCC is related to the safety level of structures. The optimum initial safety levels of structures that minimize the LCC are estimated in this paper. The structures are modeled to be subject to repeated loads.

We retrospectively studied 12 Japanese children (8 boys, 4 girls) with idiopathic membranous nephropathy (IMN), aged 2.9-15.8 (mean 7.7) years at onset. All patients were identified through either screening or a routine urinalysis; proteinuria was present in all, haematuria, which was macroscopic in 4, in 11. Three had nephrotic syndrome (NS) at or soon after onset. Stages on electron microscopy, performed in 10 patients, were I in 3, II in 5 and III in 2. Steroids alone or with cyclophosphamide were administered to 5 patients, including the 3 patients showing NS. Complete remission of proteinuria occurred in 8 patients 0.3-1.6 (mean 0.6) years after onset, and proteinuria did not recur. After a follow-up of 1.6-11.6 (mean 5.9) years, these 8 patients were in complete remission and the remaining 4 had only mild proteinuria; none had hypertension or impaired renal function. Thus, we infer that IMN in Japanese children may have a better course and outcome than IMN in non-Japanese children. Based on a comparative study of Japanese (previously reported cases added to ours) and non-Japanese (mostly Caucasian) children with IMN, this was confirmed; it is possible that steroid therapy in Japanese patients is more effective in inducing remission of NS and preserving renal function.

有意なVURの診断におけるエコー検査の有用性をprospectiveに検討した。初めて尿路感染症を発症した乳児27人 (男24人，女3人) を対象とした。エコー検査により，腎盂のballooningを4人の5腎で，尿管遠位部の4mm以上の拡張を10人の13尿管で，水腎水尿管症を2人の2腎で認めた。これらの所見を認めた10人にVCGを行い，6人の8腎にVURを認めた (IV度3腎，III度2腎，II度3腎)。ballooningを認めた5腎ではいずれもIII度以上のVURを認めた。II度の3腎のうち，2腎では尿管の拡張のみを認め，1腎では全く異常を認めなかった。VCGを行わなかった17人のうち，3人は排尿時のエコーによる観察ができず，尿路感染症が再発したのはこのうちの1人だけであり，再度のエコー検査によりballooningを，VCGによりVURを認めた。尿路感染症を発症した乳児のVURの一次診断として排尿時のエコー検査は極めて有用であった。

We explored optical excitation pathways in the multivalley semiconductor Ge in an attempt to expedite selective electron injection into the indirect L-band-edge. An off-peak resonant excitation route was developed, which offers the pumping efficiency outperforming the phonon-assisted near-indirect-edge absorption by more than six orders of magnitude. The valley selectivity results from the intra-valley relaxation that separates electrons and holes in momentum space following excitation. Fortuitously, the widely used green laser, 532 nm, is found to be nearly ideally suited to the efficient L-valley-selective excitation in Ge. Such valley-specific pumping may help clarify the otherwise complicated electron dynamics involving intervalley processes.

Several less harmful methods than voiding cysto-urethrography for detecting significant ureteric reflux have been proposed. The present prospective study investigated whether ultrasonography was effective in identifying ureteric reflux in infants with their first febrile urinary infection. The subjects were 27 infants (24 boys and 3 girls) aged from 0 to 8 months. The urinary tract was scanned when the bladder was full, and before and during induced voiding. Infants with abnormal ultrasound findings underwent voiding cysto-urethrography. The other infants were followed and those who had a recurrence of urinary infection underwent voiding cystography. Ten children underwent cysto-urethrography, with eight refluxing ureters identified in six boys. Ultrasound revealed transient dilatation of the renal pelvis on voiding in five kidneys, transient dilatation of distal ureters in 12 and hydro-ureteronephrosis in two. Each of the five kidneys with pelvic dilatation on voiding was associated with ureteric reflux grades III or IV. Of the 17 children who did not undergo cysto-urethrography, only one had recurrence of urinary infection and was diagnosed with ureteric reflux. This girl was one of the three babies who were not scanned during voiding. More than half of the infants with febrile urinary infection were excluded from invasive examination without having recurrence of urinary infection. Thus, ultrasound scanning during voiding was effective for screening infants with their first urinary infection to detect significant ureteric reflux.

Using dual-energy x-ray absorptiometry (DEXA), the lumbar spinal bone mineral density (BMD) in 49 Japanese children with or without metabolic bone disease (MBD) was determined. The following results were obtained: (a) The normal data for healthy Caucasians (J Clin Endocrinol Metab 1990; 70: 1330-1333) appear to be applicable to Japanese children; (b) BMD was normal in patients with congenital hydronephrosis with normal renal function; (c) One patient with congenital renal failure and one with Lowe syndrome had low BMD, but the MBD in the former improved markedly with peritoneal dialysis; (d) A reduced BMD was found in patients treated with long-term steroids, probably because of decreased turnover of bone; (e) A reduction in BMD was pronounced in preterm infants during the first few months of life. In conclusion, DEXA is a useful method of bone densitometry in MBD in children.

We compared several indices of proteinuria, namely protein concentration, hourly protein excretion rate (Up/h) and protein/creatinine ratio (Up/Ucr) in single voided urine samples as well as 24 h-urinary protein excretion (24 h-Up), in 44 children, aged 4–16 years, with varying degrees of urinary protein excretion. We found an excellent correlation between Up/h and Up/Ucr in early morning samples. These two indices in early morning samples had excellent correlation with 24 h-Up, comparable to those in any other urine sample of the day. Among daytime samples, Up/h varied widely, in contrast to Up/Ucr, which had significantly less variability. We analysed six early morning and six bedtime samples from 39 of these subjects, and found smaller coefficients of variation for individual patient's indices in morning samples. Up/h was more variable than Up/Ucr, especially in bedtime samples. Urinary protein concentration had a poorer correlation with 24 h-Up and was more variable than any other index. We conclude that the Up/Ucr in early morning samples, which has the advantages both of simplicity and low day-to-day variability in a given patient, is a superior index of proteinuria.

An 8-yr-old girl is presented who had periodic attacks of vomiting, psychotic depression, drowsiness, and hypertension (160/110 mm Hg) for a period of 16 months after head injury. At the initiation of the attack, serum ACTH and vasopressin levels were prominently increased (610 pg/ml and 41 microunits/ml, respectively), followed by hypercortisolemia, hyponatremia, and hypoosmolality in plasma. Serum PRL also was elevated (91 ng/ml). Responses of GH and cortisol to insulin-induced hypoglycemia and those of TSH to TRH were reduced. Urinary excretion of epinephrine and norepinephrine were increased, while dopamine (DA) excretion was reciprocally decreased, resulting in a marked elevation of the epinephrine plus norepinephrine to DA ratio during the episodes (0.4-4.5); this was normalized on attack-free days (0.08-0.25). During the attack, the concentration of homovanillic acid, a major metabolite of DA in the brain, also was reduced in cerebrospinal fluids from 70 to 23 ng/ml. The administration of methyl-dopa and reserpine effectively suppressed the recurrence of the episode. Although the exact cause of this syndrome is unknown, a periodic metabolic dysfunction of catecholamine in the central nervous system might be postulated.

Biochemical and radiographic abnormalities linked with phosphorus deficiency syndrome (PDS) developed in two very low birthweight (VLBW) infants fed exclusively with human milk. By increasing phosphorus (P) and calcium (Ca) intakes with the introduction of a specialized premature formula, osteopenia or rickets, as well as hypophosphatemia, hypo-phosphaturia and increased serum alkaline phosphatase levels improved in both patients. We speculated that all VLBW infants who are being fed exclusively with human milk should be monitored for PDS and that if PDS develops, supplementation of human milk with both P and Ca appears to be necessary for its treatment.

The bone mineral status of very low birthweight (VLBW) infants fed exclusively their own mother's milk (group I) was compared with that of VLBW infants fed mother's milk in the initial 4 weeks followed by a 1:1 mixture of mother's milk and preterm formula containing high phosphorus (P) and calcium (Ca) (group II). In both groups, most infants showed a biochemical picture characteristic of phosphorus deficiency syndrome by the fourth week. Thereafter, serum alkaline phosphatase activity (ALP) decreased and serum P increased in all group II infants. Conversely, serum ALP rose and hypophosphatemia persisted in most group I infants. Group II had a significantly higher serum P at weeks 8 and 12 and a significantly lower ALP at week 12 than group I. Furthermore, group II had a lower incidence of severe radiographic abnormalities than group I at week 12. We confirmed previous observations that VLBW infants fed exclusively human milk require P and Ca supplementation to prevent metabolic bone disease of prematurity.

Favorable course of steroid-responsive nephrotic children with mild initial attack. The course and prognosis of idiopathic nephrotic syndrome has thus far not been found to be predicted from the severity of the manifestations at the onset. Among 66 steroid-responsive nephrotic children, eight were asymptomatic without edema and identified by chance proteinuria on a urinary screening program. The selectivity index for proteinuria (a clearance ratio of IgG to transferrin) was 0.10 or less in all of the five children examined. All of the eight children responded quickly to the prednisolone therapy. Grades of proteinuria and hypoalbuminemia were lower in the asymptomatic children than in the symptomatic children who presented with edema. Median proteinuria levels were 2.0 versus 4.2 g/day/m2 (P < 0.01), respectively, and mean serum albumin levels were 2.2 ± 0.3 versus 1.8 ± 0.4 g/dl (mean ± SD; P < 0.01), respectively. None of the eight asymptomatic children relapsed for at least one year after completion of the prednisolone treatment, while, in contrast, 30 of 58 symptomatic nephrotic children relapsed during the same one-year period (P < 0.01). These findings suggest that, among steroid-responsive nephrotic children, those with mild manifestations without edema may have a milder disease and show an extremely favorable clinical course.