Molecular evidence for multicentric development of thyroid carcinomas in patients with familial adenomatous polyposis

Familial adenomatous polyposis is characterized by multiple colorectal adenomas and an increased incidence of colorectal carcinomas. Patients also develop various extracolonic tumors, of which, thyroid carcinoma is common in young females. The occurrence of multiple carcinomas in one thyroid is frequently observed, although some carcinomas are solitary. To clarify whether each carcinoma develops independently or metastatically spreads from the first one formed, we analyzed the adenomatous polyposis coli (APC) gene mutation in each carcinoma. We found that each carcinoma had a different somatic mutation of the APC gene. This is molecular confirmation for the multicentric development of thyroid carcinomas in familial adenomatous polyposis through biallelic inactivation of the APC gene.     

An increased circulating blood volume does not prevent hypotension after pheochromocytoma resection

PURPOSE: Pulse dye-densitometry, a novel monitor that measures circulating blood volume (CBV) and cardiac output (CO), was used in patients with pheochromocytoma to determine the relationship between CBV and post resection hypotension. METHODS: Case control study. An alpha blocker was administered for approximately two weeks, and its effect on the expansion of CBV was quantified. CBV was monitored in seven patients admitted for resection of suspected pheochromocytoma before preoperative alpha-blocker therapy, after alpha-blocker therapy and three times during the operation. Relationships between the CBV and blood pressure after resection of the tumour were examined. RESULTS: CBV increased from 72.0 +/- 10.0 mL.kg(-1) to 83.4 +/- 12.2 mL.kg(-1) after alpha blockade. (P < 0.001). We found a significant inverse relationship between the increase in CBV after alpha-blocker therapy and blood pressure after resection of the tumour. CONCLUSIONS: Expansion of the CBV by alpha-blocker therapy was related to lower blood pressures after resection of the pheochromocytoma. Expansion of the CBV by an alpha blocker may have increased the elastance of blood vessels. Preoperative blood volume expansion does not preclude hypotension after tumour resection. Although the CBV value itself is not a predictor for hypotension after tumour resection, pulse dye-densitometry provides values of CO and CBV simultaneously, assisting in the management of volume resuscitation and/or the need for catecholamines.     

Large contractions in the colonic J-pouch as a possible cause of incomplete evacuation

Background and aim Restoration of neo-rectal capacity is of importance in obtaining better bowel function after low anterior resection for rectal carcinoma. However, evacuatory disorders, such as incomplete evacuation, have been reported in some patients undergoing colonic J-pouch reconstruction. Therefore, we conducted this study to explore the possible factor affecting incomplete evacuation following low anterior resection for rectal carcinoma.Patients/methods The subjects were 37 consecutive patients who had undergone low anterior resection for rectal tumor (colonic J-pouch in 13 patients, straight anastomosis in 24). Clinical and physiological outcomes were determined at a mean follow-up time of 12 months after the operation, and the parameters were compared between patients with and without postoperative incomplete evacuation.Results Although anastomosis level from the anal verge was lower in the J-pouch group (6.5 cm vs 3.9 cm, P<0.05), there was no significant difference between J-pouch and straight reconstruction regarding clinical and physiological outcomes. Postoperative incomplete evacuation was significantly more frequent in the J-pouch group than in the straight group (46% vs 25%, P<0.05). Postoperative large contractions on ano-rectal manometry were also significantly more apparent in the J-pouch group than in the straight group (31% vs 4%, P<0.05). Presence of postoperative large contractions (P=0.004), anastomotic stricture (P=0.019) and smaller postoperative maximum tolerable volume (P=0.009) were significantly and independently associated with incomplete evacuation by multivariate analysis.Conclusion Colonic J-pouch reconstruction following ultra-low anterior resection was comparable with higher level straight anastomosis from the clinical and physiological point of view. The presence of large contractions might be an important indicator of incomplete evacuation in patients who are undergoing rectal resection.     

Paroxysmal kinesigenic choreoathetosis and paroxysmal dystonic choreoathetosis in a patient with familial idiopathic hypoparathyroidism

A patient with familial idiopathic hypoparathyroidism whose major symptoms were paroxysmal kinesigenic choreoathetosis (PKC) and paroxysmal dystonic choreoathetosis (PDC) was reported. CT revealed marked calcification in the basal ganglia, the thalamus and the dentate nucleus of cerebellum. Positron emission tomographic (PET) study showed decreased glucose metabolism in the basal ganglia and thalamus. The paroxysms were eliminated by the normalization of serum calcium level. Thus the paroxysms seemed to be induced by hypocalcemia which stimulated the lesions in the basal ganglia and thalamus, on one occasion, as in PKC, and on the other occasion, as in PDC. Concomitant occurrence of both PKC and PDC in a patient is unique and is of interest in considering the pathomechanisms of these different but related disorders.     

Mild hypothermia reduces expression of heat shock protein 60 in leukocytes from severely head-injured patients

BACKGROUND: Infectious complications are among the most serious problems that occur in severely head-injured patients treated with mild hypothermia. The mechanism underlying the susceptibility to infection has not been clarified. Heat shock protein (HSP) 60 has been reported to play an essential role in innate immunity. Thus, we conducted a study to clarify the impact of mild hypothermia on the expression of HSPs in polymorphonuclear leukocytes (PMNLs) in severely head-injured patients. METHODS: Between September 1997 and November 1999, 17 severely head-injured patients with a Glasgow Coma Scale score of 8 or less at admission in whom intracranial pressure could be maintained below 20 mm Hg by conventional therapy were randomly assigned to two treatment groups: a mild hypothermia group (HT group, nine patients) and a normothermia group (NT group, eight patients). The HT group was subjected to mild hypothermia (intracranial temperature, 34 degrees C) for 48 hours followed by rewarming at a rate of 1 degrees C per day for 3 days, whereas the NT group was subjected to normothermia (intracranial temperature, 37 degrees C) for 5 days. Blood samples were serially obtained at three time points; days 0 to 1, days 2 to 5, and days 6 to 14 after head injury. We measured the expression of HSP27, HSP60, HSP70, and HSP90 by flow cytometry. RESULTS: The two groups were similar with respect to prognostic factors, and there was no difference in clinical outcome. The expression of PMNL HSP60 in the HT group was significantly lower in all three time periods compared with that in the NT group (p < 0.05), whereas expression of the other HSPs did not differ significantly between the groups. The incidence of infectious complications was significantly increased in the HT group over that in the NT group (p < 0.05). In in vitro studies, PMNLs from 10 healthy volunteers were incubated at 37 degrees C, 34 degrees C, or 26 degrees C for 1 hour with sodium arsenite (100 micromol/L), an HSP inducer. The expression of HSP60 at 26 degrees C and 34 degrees C was significantly lower than that at 37 degrees C (p < 0.05), whereas expression of the other HSPs did not differ significantly at 26 degrees C, 34 degrees C, or 37 degrees C. CONCLUSION: Mild hypothermia reduces the expression of HSP60 in PMNLs from severely head-injured patients. Thus, mild hypothermia may suppress innate immunity.     

A case of miliary tuberculosis with prolonged high fever for more than 2 months under antituberculous therapy

A 28 year-old male was admitted to our hospital with persistent cough and high fever. He was diagnosed to have miliary tuberculosis by the transbronchial lung biopsy specimen and tuberculous choroidal lesions in the ocular fundus. Antituberculosis therapy was immediately started. In spite of the fact that the bacilli were sensitive to the antituberculosis drugs used and he had no other complications, high fever persisted and lasted for more than 2 months. When tuberculosis is suspected, and antituberculosis treatment is tried to observe its clinical response, the presence of similar cases mentioned above should be taken into consideration.     

A new enzymatic assay for evaluating the clearance of immune complexes from the circulation of mice.

A new photometric in vivo enzymatic immune complex clearance (EIC) assay was developed in a homologous system using glucose oxidase-anti-glucose oxidase complexes (GAG) as a model of immune complexes. Chromatographically purified GAG was injected into mouse tail veins and at intervals thereafter the enzyme activities of GAG remaining in the circulation were estimated. The GAG were cleared in a size dependent manner and were stable, being eluted as the same discrete peaks on HPLC size-exclusion chromatography both before and after injection into mice. The complement consuming activity of the GAG was weak, and depletion of complement components with cobra-venom factor did not alter clearance of the GAG from the circulation, whereas pretreatment of aggregated mouse gamma globulin suppressed the clearance rate. These results suggested that most of the GAG were not cleared via complement receptors but via FcR. Normal clearance rates were significantly changed by administration of immunomodulators such as carrageenan or LPS. Intravenous administration of GAG at a dose 50 times higher than normal caused no deaths suggesting that the complexes were of low toxicity. The enzymatic method presented should be of value for measuring the function of the mononuclear phagocytic system with respect to immune complex clearance. It provides a rapid and sensitive alternative assay which avoids using radioisotopes.     

The relationship between arginine vasopressin levels and hyponatremia following a percutaneous renal biopsy in children receiving hypotonic or isotonic intravenous fluids

Post-operative hyponatremia is a common complication in children which results from hypotonic fluid administration in the presence of arginine vasopressin (AVP) excess. We evaluated the relationship between the change in serum sodium and AVP levels following percutaneous renal biopsy in children receiving either hypotonic or isotonic fluids. This study was prompted after we encountered a patient who developed near-fatal hyponatremic encephalopathy following a renal biopsy while receiving hypotonic fluids. The relationship between the change in serum sodium and AVP levels was evaluated prior to (T0) and at 5 h (T5) following a percutaneous renal biopsy in 60 children receiving either hypotonic (0.6% NaCl, 90 mEq/L) or isotonic fluids (0.9% NaCl, 154 mEq/L). The proportion of patients with elevated AVP levels post-procedure was similar between those receiving 0.6 or 0.9% NaCl (30 vs. 26%). Patients receiving 0.6% NaCl with elevated AVP levels experienced a fall in serum sodium of 1.9 ± 1.5 mEq/L, whereas those receiving 0.9% NaCl had a rise in serum sodium of 0.85 ± 0.34 mEq/L with no patients developing hyponatremia. There were no significant changes in serum sodium levels in patients with normal AVP concentrations post-procedure in either group. In conclusion, elevated AVP levels were common among our patients following a percutaneous renal biopsy. Isotonic fluids prevented a fall in serum sodium and hyponatremia, while hypotonic fluids did not.     

The usefulness of an earphone-type infrared tympanic thermometer during cardiac surgery with cardiopulmonary bypass: clinical report

We evaluated the usefulness of a novel earphone-type infrared tympanic thermometer (IRT) during cardiac surgery with cardiopulmonary bypass. Tympanic membrane temperature (T _Tym) was monitored using the IRT inserted into the right ear canal of 12 adult patients (ASA III) who had been scheduled for elective cardiac surgery with cardiopulmonary bypass under general anesthesia. Rectum (T _Rec) and nasopharyngeal temperatures (T _Naso) were also monitored, and all temperatures were recorded at 5-min intervals during cardiopulmonary bypass. Operating room temperature was kept at 20°–27°C; a conductive warming/cooling system was used to control the patient’s body temperature. Of 265 measurements obtained, body temperature range was 31.6°–37.6°C. No complications were related to site of insertion of the monitoring probe. Significant correlations were seen between T _Tym and T _Naso (r = 0.971, P < 0.001), and T _Tym and T _Rec (r = 0.759, P < 0.001). A Bland–Altman plot showed that average temperature of T _Tym was 0.06°C above T _Naso (±0.66°C, 2 SD) and 0.12°C below T _Rec (±1.78°C, 2 SD). We conclude that an earphone-type IRT is noninvasive and hygienic and could continuously evaluate selective cerebral temperature during cardiopulmonary bypass in adults.     

Spontaneous remission in children with IgA nephropathy

Background

Some patients with IgA nephropathy (IgAN) achieve spontaneous remission even when not receiving medication. However, details on such remissions remain unknown. The aim of our study was to clarify this information in the clinical setting of childhood IgAN with minor glomerular abnormalities or focal mesangial proliferation (MGA/FMP).

Methods

This study was a retrospective analysis of 96 children with MGA/FMP who did not receive medication from among the 555 patients with newly diagnosed childhood IgAN treated between January 1972 and December 2000. The Kaplan?CMeier method and Cox proportional hazard model were used for the analysis.

Results

Of the 96 pediatric patients who did not receive medication, 57 (59.4?%) achieved spontaneous remission. The cumulative spontaneous remission rates among these patients were 57.5? and 77.4 % at 5?and 10 years, respectively, from onset. The mean time from onset to remission was 5.9?±?0.4?years. Clinical and histological findings were similar between the remission and non-remission groups. Of the 57 patients with spontaneous remissions, ten (17.5?%) also developed a recurrence of urinary abnormalities. The cumulative recurrence-free rates were 79.9?and 67.9 % at 5 and 10?years, respectively, after remission.

Conclusions

The spontaneous remission rate in childhood IgAN with MGA/FMP was higher than expected. Our results suggest that physicians should consider the potential for spontaneous remission and refrain from very aggressive treatment in IgAN patients with MGA/FMP.