A diagnosis of Menkes kinky hair disease was made in two brothers who had typical clinical symptoms and laboratory findings. The older one, 11 months old at the time of diagnosis, showed an EEG pattern of low amplitude and slow waves. Visual evoked potentials (VEPs) were absent, brainstem auditory evoked potentials (BAEPs) were abnormal. Regional cerebral blood flow (rCBF) studied by hexamethylpropyleneamine oxime single photon emission computed tomography (99mTc-HMPAO-SPECT) revealed reduced blood flow in both frontal and the right temporal regions. The younger boy, followed from birth, started seizures at the age of 3 months and had a hypsarrhythmia-like EEG. BAEPs were abnormal with prolongation of the latencies at the age of 12 months, while VEPs were near normal at 6 months, but disappeared by the age of 18 months. 99mTc-HMPAO-SPECT revealed an unexpected left parietal hyperperfusion.

Cerebrovascular Circulation↗

PubMedDate not supplied

Attenuation of Helicobacter pylori endotoxin-provoked rat intestinal inflammation by selective inhibition of the inducible nitric oxide synthase.

We studied the actions of purified Helicobacter pylori endotoxin (3 mg kg(-1), i.v.) on rat intestinal vascular permeability (assessed by the radiolabelled human serum albumin leakage technique) and on nitric oxide synthase induction (assessed by the citrulline assay) 4 h later. We found increased albumin leakage and expression of the inducible nitric oxide synthase in jejunum and colon, effects reversed by a selective inducible nitric oxide synthase inhibitor N-(8-(aminomethyl)benzyl)-acetamidine (1400W; 0.2-1 mg kg(-1), s.c., concurrently with endotoxin). Thus, H. pylori endotoxin seems to be capable of provoking an inflammatory response in the rat intestinal tissue. Systemic liberation of H. pylori endotoxin might possibly attenuate jejunal and colonic mucosal barrier function, a process mediated by the expression of the inducible nitric oxide synthase.

Amidines↗

PubMedDate not supplied

Capsaicin-sensitive mechanisms in the modulation of rat colonic vascular permeability under physiological and pathological conditions.

Inflammatory bowel disease (IBD) causes a prolonged life-quality reduction of patients and high costs for health services. The aim of this study was to explore the possible involvement of peptidergic capsaicin-sensitive afferent nerves (CSN) in the pathogenesis of IBD. For the defunctionalization of colonic CSN, the lower part of the colon (1-4 cm from the anus) was exposed through a midline laparotomy and small pieces of gelfoam moistened with a solution of capsaicin (1%, 100 microL) was applied onto the serosal surface for 30 min in male Wistar rats. Colonic vascular permeability was assessed by measuring the extravasation of [125I] human serum albumin (2 microCi/kg, i.v., 2 h prior to killing). Two months after capsaicin treatment a significant increase in albumin extravasation was found in the lower (P < 0.005), but not in the upper (5-8 cm from the anus) part of the colon as compared to the sham-operated control. Intrarectal (8 cm from anus) administration of trinitrobenzene-sulphonic acid (TNBS; 30 mg/rat) induced similar plasma leakage in the lower and upper colon of control (CSN-intact) rats (P < 0.001) 1 week later. TNBS + ethanol (50%) produced further extravasation throughout the colon (P < 0.001) of CSN-intact animals. In the lower colon of capsaicin-pretreated rats TNBS-alone provoked an increase in plasma extravasation (P < 0.001) similar to that caused by TNBS + ethanol in CSN-intact rats. In the upper colon there was no difference in the effect of TNBS-alone on plasma leakage between control (CSN-intact) and CSN-depleted rats. The results suggest that capsaicin-sensitive nerves may play a significant protective/anti-inflammatory role in the colon under normal and pathological conditions.

Albumins↗

PubMedDate not supplied

Vasopressin deficiency decreases the frequency of gastroduodenal ulceration in humans.

Vasopressin is a stress hormone released from the posterior pituitary. In humans suffering from central diabetes insipidus, this release of vasopressin is diminished. It was shown previously that the congenitally vasopressin-deficient Brattleboro homozygous rat is less sensitive to various ulcerogenic stimuli. In this study, we investigated the incidence of gastroduodenal ulceration in vasopressin deficient patients. Data on patients aged 20-70, hospitalized in Hungary between 1992 and 1995 were compared with those on the total population in this age group (6,681,020 in 1994). Subjects with central diabetes insipidus were selected separately (815 cases). Gastroduodenal ulceration was compared in subjects with an intact vasopressin release and vasopressin-deficient patients. The frequencies of gastroduodenal ulceration were also examined separately in male and female subjects. In the total population, the frequency of gastroduodenal ulceration was lower in vasopressin-deficient cases (2.22% versus 0.61%; P < 0.005). Among normal-vasopressin subjects, males have a higher risk of gastroduodenal ulceration than females (3.04% versus 1.46%, respectively; P < 0.001). Among vasopressin-deficient subjects, a similar male:female ratio was observed, but it was not significant (P = 0.36). In comparison to the normal-vasopressin population, the incidence of gastroduodenal ulceration was reduced among vasopressin-deficient males and females by 77% (P < 0.01) and by 82% (P < 0.05), respectively. In conclusion, endogenous vasopressin has a significant harmful action towards the human gastroduodenal mucosa. Peptide and non-peptide vasopressin receptor antagonists might have a potential therapeutic benefit in the treatment (as an adjuvant) and prevention of gastroduodenal ulceration.

Adult↗

PubMedDate not supplied

The results of the Putti-Platt operation with particular reference to arthritis, pain, and limitation of external rotation.

The results of 90 primary Putti-Platt operations were studied with an average follow-up of 9 years. Seventy of the patients underwent a clinical examination, a functional assessment, and radiologic examination and completed a questionnaire. Twenty patients completed only the questionnaire. The redislocation rate was 9%. Eleven percent of the patients had pain at rest, and 35% had pain on activity. Ninety percent of the patients had restriction of external rotation both at the side of the body (average loss 24 degrees) and in 90 degrees abduction (average loss 23 degrees). Twenty (29%) of the shoulders showed moderate and 1 (1%) shoulder severe osteoarthritis. Only 3 (4%) of the nonoperated shoulders showed moderate arthritis, and none had severe arthritis. Eighty-three percent of patients were fully satisfied, 13% were partly satisfied, and 4% were not satisfied with the result of the operation. The authors conclude that the incidence and severity of osteoarthritis, the incidence of pain, and the limitation of external rotation are significantly increased after the Putti-Platt operation.

Adult↗