Sign of canted ferromagnetism in the quasicrystal approximants Au-SM-R (SM = Si, Ge and Sn / R = Tb, Dy and Ho).

Magnetic susceptibility and magnetization of the quasicrystal approximants Au-SM-R (SM = Si, Ge or Sn / R = Gd, Tb, Dy or Ho) are investigated. Ferromagnetic transitions are observed in all of these compounds, in contrast to the spin-glass behavior reported in similar compounds, Ag-In-R (R = Eu, Gd, Tb or Dy). Au-SM-Gd (SM = Si, Ge or Sn) exhibit a simple ferromagnetic transition at 22.5, 13 and 9 K, respectively, whereas Au-Si-(Tb, Dy or Ho) show indications of a canted ferromagnetic transition at 8.3, 5.9 and 3.8 K, respectively. The latter are attributed to a crystal electric field effect that is absent in the Gd-bearing compounds. The ferromagnetic behavior in Au-SM-R may be understood to be a consequence of the short R-R distances compared to those for Cd-R and Ag-In-R.

Ferromagnetism and re-entrant spin-glass transition in quasicrystal approximants Au-SM-Gd (SM = Si, Ge).

Magnetic susceptibility and specific heat measurements on quasicrystalline approximants Au-Si-Gd and Au-Ge-Gd reveal that a ferromagnetic (FM) transition occurs at Tc = 22.5(5) K for Au-Si-Gd and at Tc = 13(1) K for Au-Ge-Gd, which are the first examples of ferromagnetism in crystalline approximants. In addition, a re-entrant spin-glass (RSG) transition is observed at TRSG = 3.3 K for Au-Ge-Gd in contrast to Au-Si-Gd. The different behaviors are understood based on the recent structural models reported by Gebresenbut et al (2013 J. Phys.: Condens. Matter 25 135402). The RSG transition in Au-Ge-Gd is attributed to a random occupation of the center of the Gd12 icosahedron by Gd atoms; a central Gd spin hinders the long-range FM order.

Continuous remission in an infant with chest wall malignant rhabdoid tumor after relapse.

Malignant rhabdoid tumor (MRT) is a highly aggressive tumor that occurs in infancy or childhood. The prognosis, especially in infants, is very poor. Here we report the long-term survival of a 5-month-old boy with MRT that arose from the chest wall. After total resection of the tumor, the patient was given 4 cycles of doxorubicin, vincristine, and cyclophosphamide, alternating with ifosfamide and etoposide. After 18 months off therapy, he had a local recurrence at the same site. After a second total resection, he was given additional chemotherapy with 30.6-Gy local irradiation. No further recurrence has been observed for 5 years since the second complete remission. Currently, he is alive and well at 7.5 years post-onset. Our experience in this case suggests a fundamental strategy of successful treatment of this highly malignant pediatric tumor: (1) complete resection of the localized tumor, (2) intensive multiagent chemotherapy for the minimal disseminated disease, and (3) radiotherapy for local control of the disease.

Uniform mixing of antiferromagnetism and high-temperature superconductivity in electron-doped layers of four-layered Ba(2)Ca(3)Cu(4)O(8)F(2): a new phenomenon in an electron underdoped regime.

We report (63,65)Cu- and (19)F-NMR studies on a four-layered high-temperature superconductor Ba(2)Ca(3)Cu(4)O(8)F(2)((0234F(2.0)) with apical fluorine (F(-1)), an undoped 55 K superconductor with a nominal Cu(2+) valence on average. We reveal that this compound exhibits the antiferromagnetism (AFM) with a Néel temperature T(N)=100 K despite being a T(c)=55 K superconductor. Through a comparison with a related trilayered cuprate Ba(2)Ca(3)Cu(4)O(8)F(2)(0233F(2.0)), it is demonstrated that electrons are transferred from the inner plane (IP) to the outer plane (OP) in 0234F(2.0) and 0223F(2.0), confirming the self-doped high-temperature superconductivity (HTSC) having electron and hole doping in a single compound. Remarkably, uniform mixing of AFM and HTSC takes place in both the electron-doped OPs and the hole-doped IPs in 0234F(2.0).

Uniform mixing of high-Tc superconductivity and antiferromagnetism on a single CuO2 plane of a Hg-based five-layered cuprate.

We report a site selective Cu-NMR study on underdoped Hg-based five-layered high-Tc cuprate HgBa2Ca4CU5O(12+delta) with a Tc = 72 K. Antiferromagnetism (AFM) has been found to take place at T(N) = 290 K, exhibiting a large antiferromagnetic moment of 0.67-0.69 microB at three inner planes (IP). This value is comparable to the values reported for nondoped cuprates, suggesting that the IP may be in a nearly nondoped regime. Most surprisingly, the AFM order is also detected with M(AFM)(OP) = 0.1 microB even at two outer planes (OP) that are responsible for the onset of superconductivity (SC). The high-Tc SC at Tc = 72 K can uniformly coexist on a microscopic level with the AFM at OP's. This is the first microscopic evidence for the uniform mixed phase of AFM and SC on a single CuO2 plane in a simple environment without any vortex lattice and/or stripe order.

Obstructive jaundice in a child with pancreatic hemangioma.

We report the case of a 2-year-old boy who presented with obstructive jaundice by pancreatic hemangioma. In this case, a minimal operation for obstructive jaundice without resection of the tumor was performed. Three years after the operation, the pancreatic hemangioma almost disappeared. We discuss treatment of the pancreatic hemangioma in children.

Histological investigations into the difference between cystic and fusiform types of congenital biliary dilatation.

We investigated the histological differences in the common bile duct wall of bile ducts with congenital biliary dilatation, examining 10 cystic types and 11 fusiform types. A flattened mucosa was found in the cystic type, and a tufted mucosa was occasionally found in the fusiform type. Glands in the choledochus wall were less common in the cystic type than in the fusiform type. Smooth muscle fibre was more abundant in the cystic type than in the fusiform type, and it was markedly developed in the distal choledochus in the cystic type. Based on these results we suggest that the developmental difference of smooth muscle fibres in the distal choledochus in the embryonic period may be closely related to the morphological difference between the cystic and fusiform types of congenital biliary dilatation.

Tubo-ovarian abscess after colonic vaginoplasty for high cloacal anomaly in a 13-year-old girl.

Recently a few articles have been published concerning the long-term follow-up of vaginoplasty of cloaca. However, no postoperative evaluation has been fully described and, in particular, the late complications are still unknown. We report a case of tuboovarian abscess after colonic vaginoplasty for high cloacal anomaly in a 13-year-old girl. She required a left salpingo-oophorectomy and postoperatively showed regular menstruation. Therefore we stress that tubo-ovarian abscess is one of the important late complications after colonic vaginoplasty for high cloacal anomaly.

New insight into bile flow dynamics in anomalous arrangement of the pancreaticobiliary duct.

BACKGROUND: In an anomalous arrangement of the pancreaticobiliary duct (AAPBD), two-way regurgitation, including pancreaticobiliary reflux and biliopancreatic reflux (BPR), might occur. Although pancreaticobiliary reflux has been shown by high levels of pancreatic enzymes in bile, there have been few reports of clinical evidence of BPR in AAPBD. Recently, spiral computed tomography combined with intravenous infusion cholangiography (IVC-SCT) has been reported to be useful for visualizing the biliary anatomy, which may allow a new insight into bile flow dynamics in AAPBD. METHODS: Fifteen paediatric patients with AAPBD were examined before operation using both IVC-SCT and endoscopic retrograde cholangiopancreatography. Visualization of the main pancreatic duct on IVC-SCT defined the presence of BPR. Blood samples were also analysed when the initial symptom was strongly present. RESULTS: BPR was observed in six of 15 patients. Pancreatic enlargement and hyperamylasaemia were significantly more frequent in patients without BPR than in those with BPR. Levels of enzyme markers of cholestasis were also higher in the former group. CONCLUSION: The present study, using IVC-SCT, has identified clinical evidence of BPR in AAPBD. Further studies are necessary to determine the correlation between BPR and clinical manifestations.

Relationship between the type of RET/GDNF/NTN or SOX10 gene mutations and long-term results after surgery for total colonic aganglionosis with small bowel involvement.

BACKGROUND/PURPOSE: Germline mutations of the RET-mediated or SOX10-mediated signaling pathway genes have been reported in total colonic aganglionosis (TCA). The authors investigated the possible relationship between the type of such genomic abnormalities and surgical outcomes. METHODS: Sixteen patients with TCA with extensive small bowel involvement were studied. DNA sequences of all the RET/GDNF/NTN and SOX10 coding regions were determined by the direct DyeDeoxy Terminator Cycle method. Data on the patients' clinical courses were obtained retrospectively from their medical charts and surgical records. RESULTS: RET or SOX10 germline mutations were identified in 11 of the 16 patients (68.8%). In children with aganglionosis up to the jejunum or ileum, most grew up within normal ranges, and the frequency of bowel movements decreased to 2 to 4 times per day within 5 years. However, in 5 infants with total intestinal aganglionosis, only 2 survived beyond 2 years of age, both of whom underwent Ziegler's myectomy-myotomy. A SOX10 mutation was identified in an infant with Shah-Waardenburg's syndrome, and he showed persistent bowel malfunction. CONCLUSION: The existence or type of RET mutation usually did not affect surgical results in this series of TCA patients, whereas the mutational analysis suggested 2 disease categories of TCA showing different postoperative courses, which may reflect the disparate pathogenesis in the enteric nervous system development induced by impaired RET or SOX10 signaling pathway.

Factors of biliary carcinogenesis in choledochal cysts.

The long-term complications of choledochal cysts include malignant degeneration of the epithelial lining of the biliary tract. Possible factors leading to malignant degeneration were examined by measuring amylase levels in the biliary tract, intraoperative biliary manometry and a histopathological study. 27 patients, 1 to 13 years of age, with choledochal cysts, were studied over a 13-year period. Amylase levels in the bile of choledochal cysts and the gallbladder were measured. The pressure profile of the biliary tract was recorded as the probe was withdrawn from the sphincter of Oddi (SO) to the distal end of the common bile duct. Hyperplasia of the gallbladder mucosa and malignancy of the biliary tract were investigated. Patients with high levels of biliary amylase had higher pressure differences between the SO and the duodenum than those with low levels of biliary amylase. The incidence of mucosal hyperplasia of the gallbladder mucosa was significantly higher in the fusiform type than in the cystic type. Adenocarcinoma in a cystic choledochal dilatation was found in a 12-year-old girl with high amylase levels. The cause of regurgitation of pancreatic juice into the biliary system might be due to a high pressure difference between the SO and the duodenum in addition to the lack of sphincter function at the abnormal junction of the pancreaticobiliary ductal system. Early diagnosis and surgical treatment to prevent the regurgitation leading to hyperplasia and malignancy of the biliary tract are important for children with choledochal cysts.

Intrahepatic portosystemic venous shunt associated with biliary atresia: case report.

We describe an infant with intrahepatic portosystemic venous shunt (IPSVS), which was detected by MR angiography. IPSVS is rare and its cause is disputed. However, with improvements in imaging the number of reports of IPSVS identified incidentally in patients without definite symptoms is increasing. The present case is the first associated with congenital biliary atresia and the youngest reported in the literature.

Arteriovenous malformation of the penis in an infant.

Arteriovenous malformations (AVMs) are common benign lesions in infancy, although they are rare in the genitourinary region. The authors report on a 2-year-old boy with a penile AVM. At presentation, there was venous dilatation and tortuosity of the prepuce without ulceration, which had been recognized at 1 year of age. Left internal iliac arteriography showed early venous filling leading to the diagnosis of AVM. Surgical excision was performed at age 2. The postoperative course was uneventful. At the 6-month follow-up, there was no evidence of recurrence. This is the first report of a penile AVM that was successfully treated by surgical excision.

[Sjögren's syndrome with MALT (mucosa-associated lymphoid tissue) lymphoma in a 13-year-old girl: a case report].

We report A case of Sjögren's syndrome complicated with MALT (mucosa-associated lymphoid tissue lymphoma) in childhood. Additionally, Helicobacter pylori infection into the gastric mucosa was highly suspected in this case. A 13-year-old girl suffering from multiple joint pain had received treatment as Juvenile Rheumatic Arthritis with no clinical improvement until introduction to our hospital. On admission to our hospital, high levels of serum anti-SSA and anti-SSB (80.2 and 16.1 holds, respectively) were detected. Minor salivary gland biopsy showed a typical histological finding for Sjögren's syndrome as infiltrating lymphocytes around the excretory ducts. Computed tomography and 67Gallium scintigraphy showed a gastric tumor, and it was diagnosed as primary gastric B-cell MALT lymphoma by the histopathological findings. Additionally, Helicobacter pylori infection into the gastric mucosa was highly suspected. Recently it is emphasized that infection of Helicobacter pylori is related with gastric MALT lymphoma. There has been no reports of children who have Sjögren's syndrome associated with malignant lymphoma. Taken together, Helicobacter pylori infection superimposed with Sjögren's syndrome might accelerate clinical course in our particular case.

A bleeding tendency as the first symptom of a choledochal cyst.

We report an 8-month-old male presenting with gingival hemorrhages and nasal bleeding as the first symptom of a choledochal cyst (CC). On physical examination, there was a large cystic mass in the right upper abdominal quadrant. Laboratory studies on admission revealed moderate liver dysfunction and a bleeding tendency due to vitamin K deficiency. After administration of 5 mg vitamin K the bleeding tendency disappeared. At laparotomy, a large CC 5 cm in diameter was found and the liver showed moderate cholestasis. The sudden onset of a bleeding tendency in infants with congenital liver or biliary-tract disease may suggest not only biliary atresia, but also CC.

Mutational analysis of the RET proto-oncogene in a kindred with multiple endocrine neoplasia type 2A and Hirschsprung's disease.

BACKGROUND/PURPOSE: Germline mutations of the RET proto-oncogene (RET; 10q11.2) have been reported in multiple endocrine neoplasia type 2A (MEN 2A) and Hirschsprung's disease. The authors investigated a Japanese kindred in which MEN 2A and Hirschsprung's disease frequently have been found. METHODS: The pedigree consisted of 28 members (11 boys and 17 girls) spanning 4 generations, of whom, 8 were affected with MEN 2A or Hirschsprung's disease. RESULTS: Direct sequence DNA analysis of the RET proto-oncogene showed a heterozygosity for a G to C transition at the second nucleotide of codon 620 (exon 10) in the patients, resulting in the replacement of cysteine by a serine residue in the affected Ret protein. This family added a novel RET missense mutation (C620S) predisposing to the association of MEN 2A and Hirschsprung's disease. CONCLUSION: Detection of the mutated RET gene carriers may be used for genetic counseling of potential risk for Hirschsprung's disease as well as MEN 2A in the affected families.

Mucosal cell proliferation activity of the gallbladder in children with anomalous arrangement of the pancreaticobiliary duct.

Anomalous arrangement of the pancreaticobiliary duct (AAPBD) is an anatomical maljunction of the bile duct and the pancreatic duct that is frequently associated with gallbladder carcinoma. In patients with AAPBD, it has been postulated that pancreatic juice regurgitates into the biliary tree, and the mixture of refluxed pancreatic juice and stagnant bile juice acts as an irritant factor to the biliary tract epithelium, leading to chronic inflammation and metaplasia. Eventually these mucosal changes may progress to invasive carcinoma. We reviewed clinicopathologic studies on epithelial changes of the gallbladder in patients with AAPBD to clarify the implications relevant to carcinogenesis. Conventional histological studies have shown that the most characteristic change observed in the gallbladder of children with this anomaly was epithelial hyperplasia. Furthermore, the incidence of mucosal hyperplasia was significantly increased in the gallbladder of children in whom the pancreatic duct joined the common bile duct (P-C type) compared with the incidence in children in whom the common bile duct joined the pancreatic duct (C-P type). In addition, cell kinetic studies have demonstrated increased cellular proliferative activity of the gallbladder in children with AAPBD. Cell proliferative activity was significantly elevated in children with the P-C type of AAPBD compared with that in children with the C-P type of anomaly. In conclusion, AAPBD may yield increased cell proliferation in the gallbladder of patients with this anomaly in early childhood, resulting in epithelial hyperplasia. Although it remains unknown which agents are responsible for promoting the activation of cellular function, it seems that bile acids and refluxed pancreatic proteases are likely play a role in such promotion. Further investigations are needed to elucidate the mechanism of increased cellular function.

Cellular activity in the gallbladder of children with anomalous arrangement of the pancreaticobiliary duct.

BACKGROUND/PURPOSE: Anomalous arrangement of the pancreaticobiliary duct (AAPBD) is closely related to congenital biliary dilatation and frequently associated with biliary tract malignancy. To examine the mechanism of biliary tract carcinogenesis in patients with AAPBD, we investigated histologically the early changes in cell proliferative kinetics of the gallbladder mucosa of children with AAPBD. METHODS: Twenty-three specimens of gallbladder were obtained from 23 children with AAPBD, and six control specimens were obtained from pediatric patients. All specimens were fixed routinely and paraffin embedded and examined histologically with H&E staining and immunohistochemically with monoclonal antibody Ki-67(MIB-1), which reacts with a human nuclear antigen associated with cell proliferation. Ki-67 labeling index (Ki-67 LI) was obtained by counting the numbers of Ki-67-positive cells per 1,000 gallbladder epithelial cells. RESULTS: Significant differences in Ki-67 LI were noted between children with and without AAPBD. Furthermore, Ki-67 LI and the incidence of epithelial hyperplasia of gallbladder were significantly higher in children with AAPBD in whom the major pancreatic duct joined the common bile duct (P-C type) than in those in whom the common bile duct joined the major pancreatic duct (C-P type). CONCLUSIONS: Cellular proliferative activity was increased in children with AAPBD, especially those with the P-C-type anomaly. These results suggest that the early mucosal changes of the gallbladder occurred in early childhood of patients with AAPBD and might be associated with gallbladder cancer. Early diagnosis and early surgical division of the biliary tract and pancreatic duct is recommended for children with AAPBD.

Endosonographic study of anal sphincters in patients after surgery for Hirschsprung's disease.

PURPOSE: This study was performed to evaluate the relationship between endosonographic findings of anal sphincters and anorectal function in patients after surgery for Hirschsprung's disease. In addition, whether internal sphincterotomy or sphincterectomy was accomplished was examined in each procedure by anal endosonography. METHODS: Fifteen patients, aged 11 to 20 years, were examined. Nine patients underwent modified Duhamel's operation, three underwent Rehbein's operation with anorectal myectomy, and three underwent modified Martin's operation. All 15 patients were examined by anal endosonography using a sonographic scanner (Aloka SSD 2000) with a 7.5-MHz radial rotating endoprobe, clinical bowel assessment, and manometric study. RESULTS: After the modified Duhamel operation, anal endosonography showed an interruption of the external anal sphincter (EAS) in eight of the nine patients, as a high, mixed, or low echoic lesion in the hyperechoic band correlated to the EAS. The locations of the interruption were wide in patients with mild constipation. An interruption of the internal anal sphincter (IAS) was observed in all nine patients, as a hyperechoic lesion in the hypoechoic band correlated to IAS, which indicated internal sphincterotomy. After Rehbein's operation with anorectal myectomy, an interruption of EAS was observed in all three patients as a high or low echoic lesion in the hyperechoic band. An interruption of IAS was observed in all three patients. After the modified Martin's operation, an interruption of EAS was observed in two of the three patients as a high or low echoic lesion in the hyperechoic band. An interruption of IAS was observed in all three patients. CONCLUSION: Anal endosonography showed detailed distributions of EAS and IAS in patients after surgery for Hirschsprung's disease and complete internal sphincterotomy or sphincterectomy after these operations.

Ongoing osteolysis in patients with lymphangioma.

Severe osteolysis is a rare complication of lymphangioma. It can progress despite treatment and may be fatal. Four patients presented with massive osteolysis and serious related complications. Of these, two responded well to OK-432 therapy with arrest of the osteolysis. Complete recovery from life-threatening pleural effusion has been noted in one. Of the two nonresponders, one died of progressive osteolysis of the cervical and occipital bones, the other, despite therapy, still shows progressive osteolysis of the left leg from the femur to the phalanges. A boneless leg may be the outcome.