Linear IgA bullous dermatosis of neonatal onset: case report and review of the literature.

UNLABELLED: Several small blisters were noticed on the forehead and the trunk of a newborn boy on day 1. The blisters gradually enlarged and spread over the whole body including the oral mucosa. A skin biopsy was performed twice and subepidermal bullae with polymorphonuclear and mononuclear cell infiltration were demonstrated. Direct immunofluorescence showed linear IgA, IgG and C3 depositions along the basement membrane zone and this finding led to a diagnosis of linear IgA bullous dermatosis. So far, internationally, only one case has ever been reported on the disease at neonatal onset. The skin lesions spontaneously regressed and the mucosal lesions were controlled with diaminodiphenylsulfone. CONCLUSION: In neonates with prolonged blistering, autoimmune disease such as linear IgA bullous dermatosis should be considered within the differential diagnosis and an immunofluorescence study must be performed.

Altered Mayer wave and baroreflex profiles in high spinal cord injury.

Spinal sympathetic neurons are distributed in cord segments from Th1 to L3. High spinal cord injury demonstrates severe orthostatic hypotension, but not lower cord injury. It remains to be clarified as to where is the critical spinal level disturbing neural cardiovascular regulations in response to orthostatic stress. To address this issue, beat-to-beat blood pressure (BP) (measured using a Finapres device) and RR interval (measured electrocardiographically) were recorded at rest and in a 60 degree head-up position in 26 patients with varying levels of spinal cord injury (C4 to Th12) and in 15 healthy (control) subjects. Sympathetic vascular tone was examined by the Mayer wave power spectrum of systolic blood pressure (SBP) variability. Baroreflex sensitivity was examined by transfer function analysis of SBP and RR interval variabilities. The Mayer wave power spectrum increased in response to postural shift in most patients injured at Th4 or below, whereas this parameter either remained unchanged or decreased in patients with higher-level injury. Baroreflex sensitivity tended to decrease with postural shift in patients injured at Th3 or below, whereas this parameter increased in all patients with higher-level injury. We divided spinal patients into high-level injury (Th3 or above, n = 14) and low-level injury (Th4 or below, n = 12) groups. Systolic blood pressure significantly fell (-10 +/- 4 mm Hg, P < .05) with postural shift in high-level injury group but did not change in low-level injury group or in control subjects. The low-level injury group and the control group demonstrated essentially similar autonomic nervous responses to postural shift, ie, a significant increase in Mayer wave power and an insignificant decrease in baroreflex sensitivity. On the contrary, the high-level injury group showed opposite responses, ie, an insignificant decrease in Mayer wave power and a significant increase in baroreflex sensitivity in response to postural shift. We conclude that spinal cord injury at Th3 or above eliminates normal neural cardiovascular responses to mild orthostatic stress in humans.

Sex-determining gene(s) on distal 9p: clinical and molecular studies in six cases.

We report on clinical and molecular findings in five karyotypic males (cases 1-5) and one karyotypic female (case 6) with distal 9p monosomy. Cases 1-3 and 6 had female external genitalia, case 4 showed ambiguous external genitalia, and case 5 exhibited male external genitalia with left cryptorchidism and right intrascrotal testis. Gonadal explorations at gonadectomy in cases 3 and 4 revealed that case 3 had left streak gonad and right agonadism, and case 4 had bilateral hypoplastic testes. Endocrine studies in cases 1-4 and 6 showed that cases 1, 3, and 6 had definite primary hypogonadism, with basal FSH levels of 54, 39, and 41 IU/L, respectively, whereas case 2 with severe malnutrition was unremarkable for the baseline values, and case 4 had fairly good testicular function. Fluorescence in situ hybridization and microsatellite analyses demonstrated that all cases had hemizygosity of the 9p sex-determining region distal to D9S1779, with loss of the candidate sex-determining genes DMRT1 and DMRT2 from the abnormal chromosome 9. Sequence analysis in cases 1-4 and 6 showed that they had normal sequences of each exon of DMRT1 and the DM domain of DMRT2 on the normal chromosome 9, and that cases 1-4 had normal SRY sequence. The results provide further support for the presence of a sex-determining gene(s) on distal 9p and favor the possibility of DMRT1 and/or DMRT2 being the sex-determining gene(s). Furthermore, as hemizygosity of the 9p sex-determining region was associated with a wide spectrum of gonadogenesis from agonadism to testis formation in karyotypic males and with primary hypogonadism regardless of karyotypic sex, it is inferred that haploinsufficiency of the 9p sex-determining gene(s) primarily hinders the formation of indifferent gonad, leading to various degrees of defective testis formation in karyotypic males and impaired ovary formation in karyotypic females.

Restoration of shoulder movement in quadriplegic and hemiplegic patients by functional electrical stimulation using percutaneous multiple electrodes.

The purpose of this study is to restore the motion of the paralyzed shoulder caused by upper motor neuron disorders using functional electrical stimulation (FES). Percutaneous wire electrodes were implanted into twelve muscles of the shoulder in six patients with stroke or cervical spinal cord injury. The motion of the paralyzed shoulder was controlled by a portable FES computer system, with the three standard stimulation patterns for restoring motion of 90 degrees flexion to 90 degrees horizontal abduction, 90 degrees flexion to 20 degrees horizontal adduction, and 90 degrees abduction to 90 degrees horizontal adduction. Shoulder movements were repeatedly controlled according to the created stimulation patterns in five of the patients. The two dimensional motion analyzer also confirmed shoulder control over a satisfactorily broad range of excursion. One hemiplegic patient, who was a signboard painter, had his paretic left upper extremity improved by FES, and he drew a large picture on a board with his normal right hand and, with his affected left arm against the wall, to support his trunk. This may be a world first case of producing shoulder motion through FES.

Electromyographic study relating to shoulder motion: control of shoulder joint by functional electrical stimulation.

The purpose of this study is to create the standard stimulation patterns of shoulder motion from electromyographic (EMG) data in 13 healthy human volunteers in order to control the movement of the paralyzed shoulder in quadriplegic and hemiplegic patients by functional electrical stimulation (FES). Simultaneous EMG measurement was made at 24 points of 17 major muscles relating to shoulder motion. Since the number of the output channels in the portable FES apparatus is limited, 12 major muscles were selected from statistically processing these EMG data and stimulation patterns were created based on the EMG data of these muscles. Thus three standard stimulation patterns were created to move the shoulder, i.e., (i) 90 degrees flexion to 90 degrees horizontal abduction, (ii) 90 degrees flexion to 20 degrees horizontal adduction, and (iii) 90 degrees abduction to 90 degrees horizontal adduction. With the created stimulation patterns, the restoration of the shoulder motion in plegic patients was successful and it will be reported in the next paper.

[A new reconstructive procedure, interposition of a jejunal pouch after proximal gastrectomy].

In this chapter we mention the significance or advantage of the new reconstructive procedure, interposition of a jejunal pouch after proximal gastrectomy. Instead of oesophagogastrostomy which often brought many postoperative complications, various techniques of anastomosis such as conventional jejunal interposition or double tract method were contrived. Although these techniques could reduce the incidence of postoperative problems such as reflux oesophagitis, it is very difficult to examine or remedy beyond the oesophagojejunostomy site after surgery. On the other hand, interposition of a jejunal pouch showed us many advantages. The volume of reconstructed stomach was adequate and the patients could eat enough actually. In case of need, endoscopic fiber could enter the remnant stomach or duodenum very easily. This is the big advantage for the treatment of the upper GI and hepato-biliary pancreatic diseases endoscopically or radiologically after proximal gastrectomy. For these reasons, we usually use interposition of a jejunal pouch between the oesophagus and remnant stomach after proximal gastrectomy.

Circadian blood pressure rhythm in patients with higher and lower spinal cord injury: simultaneous evaluation of autonomic nervous activity and physical activity.

OBJECTIVE: To examine the relationships among the circadian rhythms of blood pressure, autonomic nervous function, and physical activity of patients with varying levels of spinal cord injury. DESIGN AND METHODS: We studied 19 patients with spinal cord injury [10 tetraplegic patients with cervical cord injury (C4-C7), and nine paraplegic patients with thoracic cord injury (Th6-Th12)] compared with 16 control subjects. A new multibiomedical recorder was used to measure blood pressure (every 30 min), cardiac vagal activity (hourly frequency of R-R50), and physical activity (integrated acceleration/min) for 24 h under hospital conditions. Systemic sympathetic nervous activity and sympathoadrenal functioning were assessed by examination of hormone levels in the blood. RESULTS: Daytime and night-time values were compared; the variations in systolic and diastolic blood pressures and heart rate were slight in members of the tetraplegia group, but almost normal differences were observed in members of the paraplegia group. The circadian profile of cardiac vagal activity was normal for both patient groups, suggesting that an alteration in the sympathetic nervous rhythm had occurred in the tetraplegic patients. The plasma norepinephrine level was lower in members of the tetraplegia group than it was in members of the control group (P< 0.001), but was normal in members of the paraplegia group. The plasma level of epinephrine was lower in members of the tetraplegia (P< 0.05) and the paraplegia (P < 0.1) groups than it was in members of the control group. Daytime physical activity of members of both groups of patients was lower than that of subjects in the control group (P< 0.001 for both). CONCLUSION: The central sympathoexcitatory pathway to the upper thoracic cord plays a critical role in the maintenance of normal circadian blood pressure rhythm in humans. Motor nerve functioning and sympathoadrenal secretion are not essential to this regulation.

Development of an HPLC method to estimate hexosamines and its application to determine mucin content in rat and human gastric mucosa.

1. An HPLC analysis of hexosamines and related compounds modified from the Waters Pico-Tag amino acid analysis procedure is described. 2. This method is applicable to determine glucosamine and galactosamine not only in the hydrolyzate of purified glycoconjugate such as gastric mucin and ovalbumin but also in crude materials such as rat gastric mucosal extract and powdered bovine nasal cartilage. 3. Compared with a conventional colorimetric hexosamine determination, sensitivity of this method is almost 100 times higher. 4. By applying this method, a simple but highly sensitive procedure to determine mucin content in a crude stomach specimen of rat and that in a biopsied human gastric mucosa was also developed.

Stimulation of Cl- and HCO3- secretion by intramural cholinergic neurons in guinea pig antrum in vitro.

Regulation of Cl- and HCO3- secretion by intramural cholinergic neurons was investigated in guinea pig antrum in vitro. Sheet preparations composed of the mucosa and the submucosa were mounted between Ussing chambers and bathed with buffer-free solution on the luminal surface and with HCO3(-)-CO2 solution on the serosal side. Short-circuit current (Isc), unidirectional fluxes of 36Cl and 22Na, and the luminal alkalinization rate (JOHSL) were determined. Electrical stimulation of the preparations elicited increases in both JOHSL and Isc, which were inhibited by tetrodotoxin (TTX) and atropine. Physostigmine also evoked TTX- and atropine-sensitive increases in JOHSL and Isc. Similar increases in JOHSL and Isc were observed when the muscarinic agonist bethanechol chloride (BCh) was added to the serosal side. The responses to BCh were not affected by TTX. The BCh-induced increase in JOHSL was largely abolished by removal of HCO3- or Na+ and addition of ouabain (serosal side) but was neither sensitive to Cl- removal nor associated with 22Na secretion. The increase in Isc induced by BCh was associated with the increase in 36Cl secretion and was inhibited by removal of Cl- or Na+ and by addition of bumetanide or ouabain (both, serosal side). These results suggest that the submucosal cholinergic neurons are involved via muscarinic receptors in the stimulation of epithelial HCO3- and Cl- secretion. For both HCO3- and Cl-, the cellular and membrane mechanisms of secretion induced by muscarinic stimulation, although not entirely clear, appear to be different from those occurring under baseline conditions.

Effect of distal gastrectomy on gut hormone release following vagal stimulation in dogs.

The effect of distal gastrectomy on gut hormone release was investigated by electrical stimulation of the posterior truncal vagus in dogs. Peripheral and portal plasma gastrin release was significantly inhibited, but peripheral and portal somatostatin release was unchanged. Peripheral and portal pancreatic polypeptide (PP) release was significantly inhibited. Our results suggest that the following factors might be some of the reasons why the secretion of PP was inhibited after distal gastrectomy: (1) transection of the posterior vagal branches close to the antrum and pylorus which might go to the pancreas; (2) changes in other hormones such as gastrin; (3) elimination of the PP secretagogue from the gastric antrum.

Effects of bile acids and bilirubin on bicarbonate secretion of isolated guinea pig antrum.

The effects of bile acids and bilirubin, which increase in blood in obstructive jaundice, on bicarbonate secretion were studied experimentally using isolated antral mucosa of the guinea pig. Antral mucosal preparations were mounted between Ussing chambers. Basal and bethanechol-stimulated secretion were measured by a pH stat device using 5 mM HCl. After 10(-4), 10(-3), and 5 x 10(-3) M taurocholic acid, 10(-4) and 10(-3) M cholic acid, and 1.2 x 10(-4) and 2.4 x 10(-4) M bilirubin conjugate were added to the serosal solution, basal and bethanechol-stimulated secretion were also measured. Taurocholic acid, at any dose, did not affect basal secretion, but bethanechol-stimulated secretion was inhibited dose dependently. Cholic acid and bilirubin conjugate did not affect basal secretion, but bethanechol-stimulated secretion was significantly inhibited. Although the addition of bile acid or bilirubin into the serosal solution under experimental conditions is not the same as obstructive jaundice, these studies suggest that the inhibition of bicarbonate secretion in the gastric mucosa may have an important role in the formation of acute gastric mucosal lesions in obstructive jaundice.

[Plasma and tumor gastrin in patients with primary hyperparathyroidism].

It is well known that primary hyperparathyroidism is often associated with peptic ulcer. The purpose of this study is to confirm the relationship between the gastrin-levels before and after parathyroidectomy in fourteen patients with primary hyperparathyroidism, and to determine the localization of gastrin in the surgically resected parathyroid tumor. The results obtained were as follows: 1) Three patients had peptic ulcer (gastric ulcer and duodenal ulcer), the incidence being 21%. 2) The basal serum gastrin levels were 123.0% +/- 68.1 pg/ml before operation and decreased to 90.2 +/- 44.5 pg/ml after operation. In the 3 patients with slightly elevated gastrin levels, the mean level before operation was 209.1 +/- 61.2 pg/ml. The gastrin level decreased to 116.4 +/- 62.0 pg/ml after operation. 3) Gastrin immunoreactivity was detected in 10 out of 14 tumors and its localization was at the periphery of tumor cells. From these results, we conclude that extragastric gastrin secretion from parathyroid tumors may be one of the cause of peptic ulcer in patients with primary hyperparathyroidism.

Bicarbonate secretion in isolated guinea pig antrum.

HCO3- secretion was investigated in isolated guinea pig antral mucosa mounted in Ussing chambers. The rate of HCO3- secretion was measured by a pH-stat system. The antral mucosa spontaneously secreted HCO3- at a rate of about 0.5 microEq cm-2 h-1. Bethanechol (100 microM) or 8-bromoadenosine 3':5'-cyclic monophosphate (8-Br-cAMP, 1 mM) induced a significant increase in HCO3- secretion. Prostaglandin E2 (1 microM) also produced an increase in HCO3- secretion, although it was less effective than either bethanechol or 8-Br-cAMP. Histamine (100 microM) did not affect HCO3- secretion. This preparation may be suited to the study of the precise mechanisms by which neurohumoral factors regulate gastric HCO3- secretion.

Pathogenesis of water-immersion stress-induced gastric ulcers in rats with renal failure.

Rats with or without renal failure, produced by uranyl acetate, were subjected to water-immersion stress for 6 h. The severity of stress-induced gastric ulcers in the renal failure group was significantly higher than that in the control group. The intragastric pH did not significantly differ between the two groups. Transmucosal electrical potential difference, gastric mucosal blood flow, and gastric mucosal hexosamine content in the renal failure group were significantly lower than in the control group. These results suggest that decreases in defensive factors, rather than an increase in an aggressive factor, may be more closely involved in the development of acute gastric ulcers in rats with renal failure.

Experimental studies on the pharmacokinetics and therapeutic effect of cefbuperazone in biliary infection.

A study was carried out using an experimental biliary infection model to investigate the pharmacokinetic characteristics and therapeutic effect of cefbuperazone in the rabbit. Thirty rabbits were divided into three equal groups; a control group of normal animals, a group of infected animals receiving no cefbuperazone, and a group of infected animals receiving 50 mg cefbuperazone/kg intramuscularly. The experimental infection was made by direct inoculation of a suspension of E. coli into the common bile duct after ligation. The results showed that extremely high levels of cefbuperazone were achieved in bile and tissues of the biliary tract and were higher than those in the blood. Moreover, the levels were maintained at effective concentrations even after 6 hours. Viable bacterial cells from bile and the gall-bladder were barely detectable 24 and 48 hours after infection in the cefbuperazone-treated group, whilst counts remained high in the other infected group. White blood cell counts were increased at 24 hours after infection but were significantly lower in the cefbuperazone-treated group. Histological examination revealed marked inflammatory changes in the gall-bladder and bile duct of infected, untreated animals but few, mild changes only were seen in cefbuperazone-treated animals. Similarly, total bilirubin and liver enzymes were markedly increased in infected animals, but transaminases and alkaline phosphatase were significantly lower in the treated compared to the untreated group. The findings indicate, therefore, that cefbuperazone can be a useful antibiotic in biliary infection.