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[Acute reversible pan-dysautonomia: effect of erythromycin on gastrointestinal involvement].

We describe a case of acute autonomic neuropathy in an 18-year-old woman. Gut dysfunction was sufficiently severe for the patient to undergo laparotomy for suspected mechanical-intestinal obstruction before the diagnosis was made. Apart from the gut, other organs affected included the pupils, sweat and lachrymal glands. Cardiovascular autonomic function tests showed the involvement of sympathetic adrenergic nerves. Small bowel barium X-ray showed resolution of gastric stasis and emergence of jejunum dilatation during intravenous administration of erythromycin but this treatment did not eliminate intestinal obstructive symptoms. The patient had an incomplete recovery in 3 months. Erythromycin might have therapeutic value in patients with intestinal motility dysfunction in acute dysautonomia.

Acute Disease↗

Gastrointestinal manifestations of cystic fibrosis: radiologic-pathologic correlation.

Cystic fibrosis (CF), the most common lethal autosomal recessive disease in white populations, is characterized by dysfunctional chloride ion transport across epithelial surfaces. Although recurrent pulmonary infections and pulmonary insufficiency are the principal causes of morbidity and death, gastrointestinal symptoms commonly precede the pulmonary findings and may suggest the diagnosis in infants and young children. The protean gastrointestinal manifestations of CF result primarily from abnormally viscous luminal secretions within hollow viscera and the ducts of solid organs. Bowel obstruction may be present at birth due to meconium ileus or meconium plug syndrome. Complications of meconium ileus include volvulus, small bowel atresia, perforation, and meconium peritonitis with abdominal calcifications. Older children with CF may present with bowel obstruction due to distal intestinal obstruction syndrome or colonic stricture, and tenacious intestinal residue may serve as a lead point for intussusception or cause recurrent rectal prolapse. Radiologic studies often demonstrate thickened intestinal mucosal folds in older children and uncommonly show colonic pneumatosis, peptic esophageal stricture due to gastroesophageal reflux, and duodenal ulcer. Appendicitis due to inspissated secretions is uncommon. Obstruction of ducts and ductules produces exocrine pancreatic insufficiency, pancreatitis, cholestasis, cholelithiasis, and cirrhosis with portal hypertension. On imaging studies, the pancreas is commonly small and largely replaced by fat, sometimes displays calcifications, and is rarely replaced by macrocysts. Radiologic features of hepatobiliary disease include an enlarged radiolucent liver from steatosis, gallstones, a shrunken nodular liver, splenomegaly, and portosystemic collateral vessels. With the improved survival of CF patients, an increased risk for developing gastrointestinal carcinomas has been established, many occurring as early as the 3rd decade.

Adult↗

Cobalamin malabsorption due to nondegradation of R proteins in the human intestine. Inhibited cobalamin absorption in exocrine pancreatic dysfunction.

In vivo studies demonstrate that the pancreatic enzymes and the ionic environment in the upper gastrointestinal tract are essential determining factors for transport and absorption of cobalamin in man. Jejunal fluid was aspirated from healthy human volunteers after administration of cyano[57Co]cobalamin preparations. Immunochemical analysis of the aspirates demonstrated that all isotopic vitamin was transferred to a protein that is identical to the gastric intrinsic factor in terms of molecular mass (57,500), ionic nature (mean pI, 5.09), and reactivity with anti-intrinsic factor sera. However, in the aspirates from patients with exocrine pancreatic dysfunction the vitamin was found to be coupled > 60% to a protein identical to R proteins in terms of molecular mass (125,000), ionic nature (mean pI, 3.51), and reactivity with anti-R protein and anti-intrinsic factor sera. The preferential transfer of cobalamin to R proteins in the patients and to intrinsic factor in healthy subjects was associated, respectively, with low and normal levels of pancreatic enzymes in the intestine and these in turn were paralleled respectively by impaired and normal ileal absorption of cobalamin. These findings confirm the suggestion that the formation of unabsorbable cobalamin complexes may be the reason of impaired vitamin absorption in exocrine pancreatic insufficiency. Observations made with other selected patients demonstrate: (a) that decreased enzyme activity and nondegradation of R proteins may also be due to nonactivation of pancreatic zymogens in an acidic pH of the intestinal juice the vitamin transported to the jejunum couples to intrinsic factor when pancreatic function is normal, and to intrinsic factor and R protein in exocrine pancreatic insufficiency. The observations made with these selected patients may explain why not all patients with exocrine pancreatic insufficiency develop imparied cobalamin absorption, and also why the malabsorption is corrected by the administration of bicarbonate in certain patients.

Adult↗

Preliminary results of sacral neuromodulation in 23 children.

PURPOSE: Sacral nerve stimulation with InterStim is approved in the United States for use in adults. Limited data on the effectiveness of sacral nerve stimulation in children are available. We report our experience with patients who underwent InterStim placement for the treatment of severe dysfunctional elimination syndrome, which is defined as a constellation of functional urinary and gastrointestinal symptoms in patients without anatomical anomalies or obvious neurological disease, in whom intensive medical and behavioral therapies have failed to improve symptoms. MATERIALS AND METHODS: A total of 23 patients 6 to 15 years old with presenting symptoms of dysfunctional voiding, enuresis, incontinence, urinary tract infections, bladder pain, urinary retention, urgency, frequency, constipation and/or fecal soiling were followed for a mean of 13.3 months after InterStim placement. RESULTS: Of the 19 patients with urinary incontinence 3 (16%) had complete resolution, 13 (68%) had improvement, 2 (11%) were unchanged and 1 (5%) was worse (sign test, p = 0.002). Among the 16 patients with nocturnal enuresis 2 (13%) had resolution, 9 (56%) improved, 4 (25%) were unchanged and 1 (6%) was worse (sign test, p = 0.0063). Of the 15 patients with urinary retention requiring intervention 9 (60%) had improvement and 1 had worsening symptoms (sign test, p = 0.022), while 2 of 6 patients (33%) on intermittent catheterization were able to stop. One patient had development of new incontinence and enuresis. Bladder pain, urgency, frequency and constipation improved in 67% (8 of 12), 75% (12 of 16), 73% (11 of 15) and 80% (12 of 15) of the patients, respectively. Medications required postoperatively decreased by an average of 3 per patient (p < 0.001). The overall patient satisfaction rate was 64%, while that of the caregiver was 67%. Two leads were explanted from the 23 patients. Complications encountered included 1 seroma, 1 episode of skin sensitivity, 2 device failures and 1 lead revision. CONCLUSIONS: Sacral nerve stimulation in children is an option for carefully selected patients who have failed other therapies. Our results show that sacral nerve stimulation was effective in the majority of patients with the dysfunctional elimination syndrome. However, longer followup is needed.

Child↗

Ciguatera fish poisoning in the Caribbean islands and Western Atlantic.

Ciguatera fish poisoning (ciguatera), a common poisoning caused by fish ingestion, is reviewed in the Western Atlantic and the Caribbean waters. It is endemic from Florida coasts (northern limit) to Martinique Island (southern limit), with outbreaks occurring from time to time. In the Caribbean, ciguatera causes a polymorphic syndrome with gastrointestinal, cardiovascular, and neurological signs and symptoms. Neurological and muscular dysfunctions can be treated by intravenous injection of D-mannitol. The lipid-soluble toxins involved are ciguatoxins that are likely produced by the dinoflagellate Gambierdiscus toxicus. G. toxicus strains are endemic in the Caribbean Sea and in theWestern Atlantic. Although it is likely that blooms of G. toxicus are ingested by herbivorous fishes, they are not implicated in ciguatera in the Caribbean. Rather, large carnivores (barracudas, jacks, snappers, groupers), consumers of smaller benthic fish, are often involved in ciguatera. Fish toxicity depends on fishing area and depth, fish size and tissues, and climatic disturbances. Ciguatoxins have been isolated and purified from Caribbean fish species. The structure of two epimers, C-CTX-1 and C-CTX-2 from horse-eye jack, comprise 14 trans-fused ether-linked rings and a hemiketal in terminal ring. Caribbean ciguatoxins are mainly detected in the laboratory by chicken, mouse, mosquito, or cell bioassays, and by analytical HPLC/tandem mass spectrometry down to parts per billion (ppb). A ciguatera management plan that integrates epidemiology, treatment, and a simple method of detection is required to ensure the protection of consumers.

Animals↗

Plasma pancreastatin responses after intrajejunal infusion of liquid meal in patients with chronic pancreatitis.

The plasma concentrations of pancreastatin and cholescystokinin (CCK), exocrine pancreatic responses, and gallbladder contraction following intrajejunal ingestion of 100 kcal/hr semidigested liquid meal (Clinimeal) were simultaneously studied in six controls and six patients with chronic pancreatitis. An intrajejunal infusion of Clinimeal resulted in significant rises of pancreastatin and CCK, which paralleled the pancreatic secretion and gallbladder contraction. On the other hand, an intrajejunal infusion of Clinimeal resulted in a delayed rise of pancreastatin and no rise of CCK in chronic pancreatitis. Pancreatic secretion did not increase, and gallbladder contraction was not induced in these patients. It is suggested that pancreastatin may play an important role in the regulation of intestinal phase of exocrine pancreas. The impaired pancreastatin and CCK release in chronic pancreatitis may be due to the inappropriate stimuli in the lumen, which is attributed to pancreatic exocrine dysfunction, or to disturbed physiological regulation between the pancreas and gastrointestinal tract.

Adult↗

Acute hyponatremia and neuroleptic malignant syndrome in Parkinson's disease.

1. The neuroleptic malignant syndrome (NMS) may occur, occasionally, in Parkinson's disease (PD) after withdrawal of antiparkinsonian drugs. However, the circumstances in which the NMS occurs and the pathophysiologic mechanisms remain uncertain. 2. The authors studied a woman with PD, who developed hyperthermia, increased muscular tone, tremor, signs of autonomic dysfunction and stupor as symptoms of acute hyponatremia due to gastrointestinal loss of sodium in excess of water. 3. The correction of hyponatremia led to a complete recovery after about 6 hours. During this period the antiparkinsonian therapy was not modified. 4. An acute imbalance of sodium in the central nervous system may play a role in the pathophysiology of NMS.

Aged↗

Effect of pelvic radiotherapy for prostate cancer on bowel, bladder, and sexual function: the patient's perspective.

OBJECTIVES: To document the effects of pelvic radiotherapy on bowel, bladder, and sexual function, as reported by the patient. METHODS: A confidential questionnaire was distributed to 202 prostate cancer patients. Mean age was 70 years (range, 49 to 87) and mean follow-up was 33 months (range, 12 to 72). Radiation was given by a standard four field box technique on a linear accelerator to 60 to 66 Gy over 6.5 weeks. Treatment was limited to the prostate and seminal vesicles for small well-differentiated tumors, but otherwise included internal and external iliac nodes. RESULTS: Responses were obtained from 192 patients (95%). No or mild change in bowel function was reported by 64% of patients, 25% reported moderate change, and 11% reported severe change. Rectal urgency was a concern for 20%, and 5% reported fecal soiling in the preceding 6 months. Hematochezia at least once a week was reported by 9% and daily by 5%. Frequent hematochezia decreased from 17% in the second and third post-treatment years to 4% after 3 years (P = 0.02). Transfusions or laser surgery for proctitis was required by 4%. No patient required a colostomy for rectal complications. Urinary stream was unchanged or improved for 83%. Nocturia was unchanged or improved in 70%. Some form of protection for urinary incontinence was required by 2%, and 0.5% noted frequent hematuria and 2% moderate to severe dysuria. Potency prior to radiotherapy was reported by 82% and was lost following radiotherapy in 35%. Technical factors, including treatment volumes and total dose, did not correlate to the risk of moderate or severe complications. CONCLUSIONS: The sequelae of pelvic radiotherapy as viewed from the patient's perspective reveal a major alteration in bowel function in 11%, significant bladder symptoms in 4%, and loss of potency in 35%.

Aged↗

Amyloid goiter in cystic fibrosis.

Chronic infectious or inflammatory diseases lead to amyloid infiltration and dysfunction of many organs, including the kidney, liver, heart, and gastrointestinal tract. Subclinical amyloid infiltration of the thyroid gland has been described in over 80% of such patients. However, symptomatic involvement of the thyroid gland by amyloid is rare. We describe a euthyroid patient with cystic fibrosis and widespread amyloidosis who presented with a rapidly enlarging goiter and symptoms of local compression that compromised his pulmonary status. Fine needle aspiration of the goiter was nondiagnostic. At surgery he proved to have replacement of the thyroid gland by amyloid. A review of the literature reveals only five previous cases of amyloid goiter in patients with cystic fibrosis. However, as more patients survive into adulthood, amyloid goiter may become a more common complication of cystic fibrosis. In contrast to other patients with reactive amyloidosis and goiter, patients with cystic fibrosis may require thyroid surgery to relieve airway compression that can compromise pulmonary function.

Adult↗

Effects of dietary acidification and potassium depletion on acid-base balance, mineral metabolism and renal function in adult cats.

Effects of dietary potassium restriction, with or without dietary acidification, on acid-base balance, mineral metabolism and renal function were evaluated in 12 adult cats. Six cats were fed a potassium-restricted diet (0.2% potassium) for 8 wk, and six cats were fed the same potassium-restricted diet plus a dietary acidifier (0.8% NH4Cl) for 8 wk. Both groups of cats were then fed the same diet supplemented with potassium gluconate (0.7% dietary potassium) for an additional 4 wk. Renal function was evaluated before treatment and again at 8 and 12 wk. Serum potassium concentration declined in all cats by wk 1 and was also lower in NH4Cl-treated cats at 2, 3, 6 and 8 wk than in control cats. Metabolic acidosis developed in both groups of cats. Dietary balance studies indicated negative potassium balance in NH4Cl-treated cats. Glomerular filtration rate declined significantly in NH4Cl-treated cats after 8 wk but was unchanged in control cats. From the results of this study, we conclude that adding a dietary acidifier to a potassium-restricted diet worsens hypokalemia, possibly by affecting gastrointestinal potassium handling, and induces severe metabolic acidosis and renal dysfunction in adult cats.

Acidosis, Renal Tubular↗

Cadmium exposure and nephropathy in a 28-year-old female metals worker.

A 28-year-old female presented for evaluation of left flank pain and polyuria after having been exposed to cadmium in the jewelry manufacturing industry for approximately 3 years. This patient possessed both elevated 24-hr urinary ss2-microglobulin and elevated blood cadmium levels. Approximately 6 months after initial presentation, the patient resigned from her job due to shortness of breath, chest pain, and anxiety. Exposure to cadmium in the jewelry industry is a significant source of occupational cadmium exposure. Other occupational sources include the manufacture of nickel-cadmium batteries, metal plating, zinc and lead refining, smelting of cadmium and lead, and production of plastics. Cadmium is also an environmental pollutant that accumulates in leafy vegetables and plants, including tobacco. Major toxicities anticipated from cadmium exposure involve the renal, pulmonary, and, to a lesser extent, gastrointestinal systems. These include the development of renal proximal tubular dysfunction, glomerular damage with progressive renal disease, and respiratory symptoms including pneumonitis and emphysema. Low-level cadmium exposure has also been associated with increased urinary calcium excretion and direct bone toxicity, effects that recent research suggests may result in the development of osteoporosis. The body burden of cadmium, over half of which may reside in the kidneys, is most often measured through the use of urinary cadmium levels. Blood cadmium measurements generally reflect current or recent exposure and are especially useful in cases with a short exposure period and only minimal accumulation of cadmium in the kidneys. Both ss2-microglobulin and alpha1-microglobulin serve as organ-specific, early-effect biomarkers of tubular proteinuria and thus play a role in identifying early signs of cadmium-induced renal damage in those with potential exposures. In addition to ensuring workplace compliance with Occupational Safety and Health Administration-mandated monitoring and screening measures, it is prudent for those with cadmium exposure to maintain adequate intake of both iron and calcium, appropriate measures even in the absence of exposure.

Adult↗

Alcohol and the small intestine.

Several alterations of the small-intestinal morphology and function have been documented after alcohol ingestion. There are morphologic changes macroscopically and microscopically after acute alcohol administration in the proximal part of the small intestine, which are quickly reversible. There are no macroscopic changes and, in most patients, very discrete light microscopic changes in the small intestine after chronic alcohol ingestion. The ultrastructural changes are, however, profound, as seen by both transmission and scanning electron microscopy. The permeability is probably increased, permitting entrance of possible noxious agents, which may explain some of the extraintestinal tissue damage observed in chronic alcoholism. The transit is increased, at least after acute alcohol administration, perhaps contributing to the diarrhea commonly seen after heavy drinking. Several of the enzymes located in the brush border are affected; lactase activity can be depressed and perhaps result in a transient milk intolerance in predisposed individuals. The activity of GGT is increased and may partly account for the GGT elevation in serum after heavy drinking. Other enzymes, such as Na(+)-K(+)-ATPase, can be inhibited and result in a decreased absorption of substances that require active, energy-dependent transport mechanisms. The secretion of water and electrolytes may be increased (an effect on cAMP?). The absorption of several nutrients, vitamins, and other elements is disturbed. The bacterial flora is increased and changed, which may give rise to symptoms and also increase the production of acetaldehyde by bacterial metabolism of ethanol. Acetaldehyde is more toxic than ethanol, and an increased concentration of acetaldehyde can possibly accentuate the damage to the liver and other organs. The bacterial overgrowth can possibly cause endotoxinemia. Although studies on alcohol-related intestinal alterations have been relatively sparse, the acute and chronic effects of alcohol thus seem to be considerable. From available results it is, however, difficult to draw any definite conclusions about the clinical importance of many of the findings. Future research will need to study the relationship between, for example, the chronic morphologic changes, the absorptive dysfunction, and permeability alterations, on the one hand, and the gastrointestinal symptoms, the extraintestinal damage, and various deficiencies, on the other hand.

Animals↗

Perhexiline maleate in the treatment of severe angina pectoris.

Perhexiline maleate was used as a prophylactic agent in 26 patients suffering from severe angina pectoris. The mean duration of treatment was 8.9 months, with a maximum of 28 months. Fifteen patients experienced a reduction in frequency of attacks to less than one-third of their previous level; six experienced a reduction to two-thirds of their previous level; no patient showed an increase in attack rates. During the period of study, there was one death. Frequently observed side effects included dizziness, gastrointestinal irritation and malaise. One patient developed clinically apparent hepatic dysfunction which resolved on withdrawal of perhexiline maleate, but recurred after rechallenge with a lower dose of the drug; the results of liver function tests in five others showed mild abnormalities. One patient developed peripheral neuropathy after taking perhexiline maleate for 18 months, but this resolved in two months after cessation of therapy. Good responses to perhexiline maleate were observed in patients who were concurrently treated with beta-adrenoreceptor blocking drugs.

Alprenolol↗

[Urethral lengthening with a tube flap (Kropp's operation) and gastrocystoplasty].

Gastrocystoplasty associated to a Kropp procedure in our experience has been an excellent alternative to improve the quality of life and renal function in patients with severe bladder dysfunction and urinary incontinence. Technically offers easy development of a gastrointestinal segment not requiring ureteral reimplantation or detubularization with the consequent excess of sutures required traditionally in these procedures. Also the postoperative care was smoother and shorter than previously using with the obvious advantage of less mucus production and no metabolic disturbances often present. Further catheterization might be performed through a physiological way obviating abdominal stomas.

Adolescent↗

[Acute abdomen states in geriatric patients in the intensive care unit complicated by hypovolemic shock].

UNLABELLED: Elderly patients suffering from acute pathology and severe medical comorbidities present serious "mix-cases" and their multiple organ deterioration requires intensive care. GOAL OF THE STUDY: analysis of outcome of elderly patients, aged 65 years and over, suffering from acute abdominal surgical pathology associated with hypovolemic shock. MATERIALS AND METHODS: 2 244 patients aged 65 yrs and over were treated with an acute abdomen state in the 2nd Chair and Department of Surgery in Jagiellonian University Hospital within 01.07.1997 and 30.06.2004. 618 patients, aged > 65 yrs underwent surgery because of acute abdominal pathology with hypovolemic shock. Out of this group 239 cases presented peritoneal diffuse inflammation and 104 were diagnosed with bowel obstruction. The principles of the diagnosis and the therapy were common for both groups. The analysis included: mortality rate, duration of hospital and intensive care unit (ICU) stay, continuos assessment based on APACHE II (Acute Physiology And Chronic Health Evaluation) and TISS-28 (Therapeutic Intervention Scoring System) scales, and on modification of MODS (Multiple Organ Dysfuction Score) system. RESULTS: mortality rate patients with peritoneal diffuse inflammation was 51.05%, and was higher than patients with bowel obstruction 48.08%. The difference in these values was caused by infection by pathogenic bacteria of gastrointestinal tract and most frequently by the appearance of multiorgan dysfunction in the group with peritoneal diffuse inflammation, despite lower punctation in the APACHE II and TISS-28 score system.

Abdomen, Acute↗