L-carbocisteine can cause cholestasis with vanishing bile duct syndrome in children: A case report.

RATIONALE: Vanishing bile duct syndrome (VBDS) is the acquired progressive destruction and disappearance of intrahepatic interlobular bile ducts in the absence of underlying liver or biliary tract disease, causing chronic cholestasis. Infections, drugs, toxins, malignant diseases, and certain immunological processes are associated with the development of this syndrome. There have been no reports of children developing VBDS as a consequence of the administration of L-carbocisteine. PATIENT CONCERNS: A 9-year-old Japanese girl presented with fever, jaundice, and skin rash. Laboratory investigations revealed elevated levels of serum transaminases, γ-glutamyltransferase, and bilirubin. Histopathological features were consistent with a diagnosis of VBDS. Drug-induced lymphocyte stimulation tests (DLST) were positive for L-carbocisteine. DIAGNOSIS: VBDS caused by L-carbocisteine. INTERVENTIONS: Ursodeoxycholic acid and discontinuation of L-carbocisteine. OUTCOMES: The patient responded to treatment based upon discontinuation of L-carbocisteine and administration of ursodeoxycholic acid. Her transaminase and bilirubin levels were normalized gradually. LESSONS: Physicians should be aware of the fact that L-carbocisteine can cause cholestasis with VBDS in children.

Child requiring tracheostomy for removal of an airway foreign body at the tracheal bifurcation.

Airway foreign bodies are typically removed orally using a rigid bronchoscope. We present a rare case of a foreign body at the tracheal bifurcation that required removal via tracheostomy. A child turned pale while eating nuts and was suspected to have choked on a foreign body. CT revealed a foreign body at the tracheal bifurcation. As his respiratory condition was unstable, tracheal intubation and removal were attempted using a rigid bronchoscope. Tracheal obstruction during oral removal resulted in respiratory failure and bradycardia. Following emergency tracheostomy, the foreign body was removed via the tracheal stoma after his respiratory condition stabilised. The patient was discharged 21 days later without neurological sequelae. To avoid hypoxaemia during airway foreign body removal, as in this case, assessing the size of the upper airway and foreign body is necessary. Tracheostomy and foreign body removal through the tracheal opening should be considered proactively.

Function of the head-tail junction in the activity of myosin II.

All class II myosins have the conserved amino acid sequence Pro-Leu-Leu at their head-tail junctions. We systematically altered this sequence in smooth muscle heavy meromyosin (HMM) by site-directed mutagenesis and examined the effects of these mutations on actin-myosin interactions. Deletion of the proline and second leucine did not cause any noticeable change in either actin-activated ATPase activity or actin-sliding velocity. In contrast, deletion of the two leucine residues and substitution of the first leucine with alanine resulted in a 14-fold and 5-fold decrease, respectively, in actin-activated ATPase activity. However, both these mutations did not appreciably affect actin-sliding velocity, which was consistent with a result that there was no considerable change in the ADP release rate from acto-HMM in the deletion mutant. In contrast to double-headed HMM, a single-headed subfragment-1 (S1) with a Leu-Leu deletion mutation exhibited actin activated ATPase activity similar to that by wild type S1. Our results suggest that the first leucine of the conserved Leu-Leu sequence at the head-tail junction profoundly affects the cooperativity between the two heads involved in the actin activated ATPase activity of myosin II.

Ruptured mucinous cystic neoplasm with an associated invasive carcinoma of pancreatic head in a pregnant woman: report of a case and review of literature.

Mucinous cystic neoplasm (MCN) of the pancreas is characterized by mucin-producing columnar epithelium and an ovarian-type stroma. It occurs almost exclusively in women and is almost always located in the pancreatic body or tail. Here, we report a case of large MCN located in the pancreatic head but not in the body nor tail in a 32-year-old pregnant woman, which was thought to have grown rapidly during pregnancy. It was ruptured at 34 weeks of gestation and the patient was admitted to the emergency department of the University of Fukui Hospital with an acute abdomen. Emergency cesarean section followed by pancreaticoduodenectomy was performed. The tumor consisted of many small cysts lined by a single-layer of mucinous epithelium with papillary growth and partial solid parts showing invasive growth and sarcomatoid changes, indicating mucinous cystic neoplasm with an associated invasive carcinoma (previously referred as mucinous cystadenocarcinoma). Thickened septa revealed ovarian-type stroma strongly positive for α-inhibin and partly positive for progesterone receptor immunohistochemically. We also review and discuss previous reports of MCNs including those with an associated invasive carcinoma in pregnant patients.

The effectiveness of transverse coloplasty in patients with ultra-lower rectal cancer.

Rectal cancer accounts for 40% of colon cancer, and postoperative defecatory function is considered to markedly affect the patients' quality of life. We performed transverse coloplasty in 33 patients with rectal cancer who had undergone an anal function preservation operation in which the anastomotic site was within 1 cm of the dentate line (ultra-low anterior resection) and evaluated its effectiveness in controlling the patients' defecatory function. The average daily defecation frequency 1, 6, and 12 months postoperatively was 7.8, 5, and 3.6 times daily following straight colorectal reconstruction (the anastomotic site was more than 5 cm from the dentate line) and 7.5, 3.5, and 2.4 times daily following transverse coloplasty, respectively. Concerning postoperative complications, anastomotic leakage, soiling, and constipation were observed in 1, 1, and 1 cases, respectively. Transverse coloplasty can be performed in a short time, and it is considered a safe and useful method to manage defecatory

Biliopancreatic fistula and abscess formation in the bursa omentalis associated with intraductal papillary mucinous cancer of the pancreas.

We describe an unusual case of biliopancreatic fistula, free perforation, and subsequent abscess formation within the lesser peritoneal sac associated with intraductal papillary mucinous carcinoma (IPMC). A 71-year-old man presented with general fatigue and loss of appetite that had persisted for 1 month. Abdominal computed tomography (CT) revealed findings consistent with an intraductal papillary mucinous neoplasm (IPMN) of the pancreas, accompanied by abscess formation in the bursa omentalis. Gastrointestinal fiberscopy revealed a swollen papilla of Vater expanded by sticky mucus, and a communication between the pancreatic duct and bile duct was demonstrated by the injection of indigo carmine solution into the pancreatic duct. Percutaneous transhepatic abscess drainage (PTAD) was performed on the day of admission. After this procedure, the patient was managed for 1 month and supported nutritionally with glycemic control for diabetes mellitus. After admission, the patient had an episode of obstructive jaundice that was treated by retrograde biliary drainage. Pancreaticoduodenectomy with lymph node dissection was then performed. Pathological examination revealed IPMN with patchy, scattered carcinoma of the pancreatic head and uncinate process with the formation of a biliopancreatic fistula. Bile duct epithelium in the area of the biliopancreatic fistula demonstrated atypical papillary epithelium suggestive of tumor invasion.

Chemo-hyperthermic peritoneal perfusion (CHPP) for appendiceal pseudomyxoma peritonei.

BACKGROUND: Pseudomyxoma peritonei is derived mostly from the rupture of an appendiceal cystic tumor and produces a large quantity of mucinous substance. Though its malignancy level is low, radical resection is difficult and its prognosis is poor. In some institutes in European countries and the United States, multidisciplinary treatment with chemo-hyperthermia at a relatively low temperature is performed following subtotal peritonectomy. We carried out high-temperature chemo-hyperthermic peritoneal perfusion following incomplete resections of mucinous tumors in six patients. METHODS: After resection of the main tumor and macroscopic gross tumor resection of dissemination, heated perfusate containing anticancer agents was poured into the peritoneal cavity and stirred and pumped into a circulation between the abdomen and a reservoir. The temperature of the surface of the peritoneum was maintained at around 43 degrees C. RESULTS: The intraperitoneal space was sufficiently heated without severe complications. The pathological diagnosis in four of the patients was peritoneal mucinous carcinomatosis, and in the other two patients, it was intermediate type between peritoneal mucinous carcinomatosis and disseminated peritoneal adenomucinosis. Two patients died, 15 and 26 months after the treatment. The other four patients have survived for 4, 24, 26, and 80 months now, and two of them, who have survived for 26 and 80 months, received a second treatment 18 and 32 months, respectively, after the initial treatment. These results seemed not to be inferior to those in the reports from institutes in Europe and the United States which performed subtotal peritonectomy and then used mild hyperthermia in chemo-hyperthermia. CONCLUSION: For treating pseudomyxoma peritonei, high-temperature chemo-hyperthermic peritoneal perfusion following incomplete tumor resection is effective even without peritonectomy.

Aurora-B associated protein phosphatases as negative regulators of kinase activation.

The human serine/threonine kinase Aurora-B is structurally related to the protein kinase Ipl1p from S cerevisiae and aurora from Drosophila melanogaster, which are key regulators of mitosis. The present study shows that human Aurora-B is activated by okadaic acid and forms complexes with the protein serine/threonine phosphatase type 1 (PP1) or PP2A, but not with PP5. These data identified Aurora-B associated protein phosphatases as negative regulators of kinase activation. We then used a series of substrates based on a histone H3 phosphorylation site (residues 5-15) to determine the substrate specificity of human Aurora-B. We found that this enzyme is an arginine-directed kinase that can phosphorylate histone H3 at serines 10 and 28 in vitro, suggesting that human Aurora-B is a mitotic histone H3 kinase.

Intraductal mucinous tumors occurring simultaneously in the liver and pancreas.

A case of simultaneous intraductal mucinous tumors of the liver and pancreas in a 67-year-old man is described. Abdominal ultrasonography and computed tomography (CT) revealed the presence of cystic lesions with intraluminal septae both in the caudate lobe of the liver and in the uncinate process of the pancreas; these cystic lesions communicated with the hepatic duct and pancreatic duct, respectively. Mucin retention was observed in the cysts, and cholestasis was induced by mucin secretion into the common bile duct. The lesions were resected by left hepatic lobectomy with caudate lobectomy, and segmental pancreatectomy. Both lesions were multilocular cystic tumors with no papillary projections or focal mass effect in their walls. Histologically, both cystic lesions were a mixture of hyperplasia and adenoma lined by low papillary columnar epithelium. There were no cellular or histological features to suggest malignant change. The fibrous intratumor interstitium lacked any mesenchymal or ovarian-like stroma. The hepatic lesion was considered to be of a similar nature to intraductal papillary mucinous tumor (IPMT) of the pancreas. However, the two lesions occurred simultaneously in the liver and pancreas. This case is of interest in regard to the diagnosis and management of mucinous hepatopancreatobiliary lesions.