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PubMed1984

Dumping syndrome following Nissen's fundoplication: a cause for refusal to feed.

Two cases of infantile dumping syndrome which developed following Nissen fundoplication for gastroesophageal reflux are described. Both infants were fed postoperatively via a gastrostomy and showed the typical clinical picture of dumping with failure to thrive, intermittent diarrhea, lethargy and pallor postprandially. Several glucose tolerance tests were highly pathological with marked hyperglycemia immediately after a gastrostomy meal followed by hypoglycemia two hours later. In one case HbA1c was significantly elevated which is thought to be an expression of recurrent hyperglycemia. In both infants the first and most impressive clinical sign was absolute refusal or oral feeds. Normal oral food intake was slowly re-established after normalization of blood glucose homeostasis.

Dumping Syndrome↗

PubMed1975

Glycerol intolerance in a child with intermittent hypoglycemia.

A 3-yr-old boy was investigated for numerous episodes of fatigue, irritability, pallor, and sweating, which began at 11 mo of age, when he had an episode of symptomatic hypoglycemia with ketonuria. He had euphoria, mental confusion, drowsiness, nausea, and vomiting 1-5 hr after oral administration of glycerol in doses of 0.5-1.0gm/kg. Orally administered MCT (1 gm/kg) had similar effects. On one occasion, oral glycerol also provoked hypoglycemia, as had a 16 1/2 hr fast. Intravenously administered glycerol (0.09 gm/kg) induced an immediate loss of consciousness from which he recovered spontaneously after 30 min; there were no changes in blood glucose values. Intravenously administered fructose (0.25 gm/kg) was tolerated normally. Leukocytes showed normal activities for FDPase, glycerol kinase, and glycerol phosphate dehydrogenase. The restriction of dietary intake of fat has been associated with a marked improvement in physical and mental activities. These observations suggest a unique, yet undifined intolerance to glycerol, which suggest caution in the diagnostic use of glycerol in the investigation of hypoglycemia as well as in the therapy of increased intracranial or intraocular pressure.

Alanine↗

PubMed1986

Manifestations of milk allergy in infancy: clinical and immunologic findings.

In a study of the manifestations of cow milk allergy in 100 young children (mean age 16 months), 30 items of historical data and information relating to the effects of a standardized milk challenge were entered into a computer data base. Three clusters of patients were derived using a K-means algorithm. In group 1 were 27 patients with predominantly urticarial and angioedematous eruptions, which developed within 45 minutes of ingesting cow milk. They had positive skin test reactions to milk and elevated total and milk specific IgE serum antibody levels. In group 2, 53 patients had pallor, vomiting, or diarrhea between 45 minutes and 20 hours after milk ingestion. These children were relatively IgA deficient. The 20 patients in group 3 had eczematous or bronchitic or diarrheal symptoms; in 17 symptoms developed more than 20 hours after commencing milk ingestion. Of the patients in group 3, only those with eczema had a positive skin test reaction and elevated IgE antibodies to milk. The patients in group 3 were the most difficult to identify clinically; they had a history of chronic ill health, and symptoms developed many hours or days after commencing milk ingestion in the challenge situation. In view of the heterogeneous clinical and immunologic findings in our patients, it is unlikely that a single laboratory test will identify cow milk allergy in all susceptible patients.

Angioedema↗

PubMed1999

Spontaneous arterial thrombosis in children.

Spontaneous peripheral artery thrombosis in children is rare. We present 2 cases, in both of which the diagnosis was delayed. Acute arterial insufficiency should be considered in children who have clinical symptoms of leg pain, pallor, and reduced pulses. Angiography is the gold standard to confirm or exclude the diagnosis.

Aspirin↗

PubMed1999

White matter dementia in CADASIL.

Cerebral white matter disorders may be associated with profound neurobehavioral dysfunction. We report a 62-year-old man who had a slowly progressive 25-year history of personality change, psychosis, mood disorder, and dementia. Neurologic examination disclosed abulia, impaired memory retrieval, and preserved language, with only minimal motor impairment. Neuropsychological testing found a sustained attention deficit, cognitive slowing, impaired learning with intact recognition, and perseveration. Magnetic resonance imaging of the brain revealed extensive leukoencephalopathy. Right frontal brain biopsy showed ill-defined white matter pallor with hyaline narrowing of white matter arterioles. Granular osmiophilic material adjacent to vascular smooth muscle cells on electron microscopy of a skin biopsy, and an arginine for cysteine replacement at position 169 in the 4 EGF motif of the notch 3 region on chromosome 19q12 established the diagnosis of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). This case illustrates that CADASIL can manifest as an isolated neurobehavioral disorder over an extended time period. The dementia associated with CADASIL closely resembles that which may occur with other white matter disorders, and represents an example of white matter dementia.

Brain↗

PubMed2000

Pyruvate dehydrogenase activity and energy metabolite levels following bilateral common carotid artery occlusion in rat brain.

The influence of chronic cerebral hypoperfusion on cerebral energy metabolism was studied. The bilateral common carotid arteries of Wistar rats were occluded for 0, 2, 7, and 28 days. Cerebral energy metabolism was evaluated by assaying adenosine triphosphate (ATP), phosphocreatine (PCr), and lactate levels and measuring pyruvate dehydrogenase (PDH) activity (each time point, n = 6). Pathological changes were assessed light-microscopically by Klüver-Barrera staining and immunohistochemical labeling for astroglia (each time point, n = 3). There were no changes in ATP and PCr levels or PDH activity; there was slight but significant transient lactate accumulation at 2 days. Myelin pallor and increase in immuno-reactive astroglia were only observed at 28 days. These results indicate that chronic cerebral hypoperfusion induces delayed white matter changes in the corpus callosum of rat brain, but does not affect energy production.

Adenosine Triphosphate↗

PubMed1976

Renal hypertension in children.

Preliminary results of this retrospective-prospective analysis of renal hypertension in 110 children indicate that hypertension may be secondary to a wide variety of acute progresive, and chronic renal diseases which may be either congenital or acquired. Affected children may be detected at any time from infancy through adolescence. Symptoms usually associated with acute glomerulonephritis (i.e., headache, swelling, nausea, vomiting, anorexia, fatigue, dizziness, and fever) occur in both acute and chronic renal diseases associated with hypertension. Headache and swelling are the most common symptoms in this series. Peripheral edema, rales, and increased heart size were found in between 10 and 25% of these children. Differential diagnosis may be approached by a consideration of causes of acute and chronic hypertension. The child with chronic renal disease usually presents with a long history of fatigability, poor growth, and pallor, and laboratory tests reveal elevation of the creatinine and BUN along with anemia, hypocalcemia, and hyperphosphatemia. In contrast, the child with acute renal disease and hypertension presents with a history of prior good health followed by the abrupt onset of signs and symptoms of renal disease; laboratory tests usually reveal modest elevations of creatinine and BUN, anemia is unusual, an abnormal urinalysis is common, and serum calcium and phosphorous levels are usually normal. Renovascular and asymmetric renal parenchymal disease represent uncommon but important conditions because surgery may be curative. Treatment may be surgical, medical, or combined. Surgical conditions include renal trauma, hydronephrosis, asymmetric renal disease, and renal arterial disease. Adequate blood pressure control without medication can be expected following surgery in instances of unilateral involvement with a normal contralateral kidney. Meticulous assessment of the contralateral kidney is needed to determine that it is normal. If surgery is unsuccessful or is not indicated, pharmacologic therapy is initiated with a stepwise regimen starting with the mildest agent (e.g., thiazides) and then adding additional antihypertensive drugs when adequate blood pressure control has not yet been achieved. The goal of therapy is the lowest, safest, tolerated blood pressure levels. Long-term, carefully designed studies of antihypertensive agents for children with renal hypertension are not available. The need for collection and critical analysis of data concerning the clinical course of children with renal hypertension is evident from a review of the literature and from the preliminary data presented in this series. The presentation of such information and a critique of outcome variables will provide a basis for program planning for affected children and improvement in patient care where indicated.

Adolescent↗

PubMed1986

Anemia of prematurity. Current concepts in the issue of when to transfuse.

At no other time of life is the decision to transfuse potentially as difficult as in the newborn period. Superimposed upon complex "physiologic" changes in the ability to deliver and release oxygen are varying requirements among infants in terms of oxygen need. These are compounded by changes brought about as a direct consequence of frequent phlebotomy in the most ill of preterm infants. Despite the confusion overlying many of the changes occurring at this time of life, certain principles can be applied. Unlike that of the adult, an infant's ability to make oxygen available in response to a specific demand is almost as dependent upon the modifiers of oxygen uptake and release by hemoglobin as upon the hemoglobin concentration itself. These modifiers are constantly changing, sometimes in a predictable fashion, sometimes not. As discussed, some attention to the status of a particular infant's capability in providing oxygen relative to need will assist in the decision when to transfuse. If specific parameters of these assessments can not be determined, it may be necessary to proceed with transfusion based on the clinical presentation of an infant. With regard to the above, any infant sufficiently ill to require frequent blood sampling should have such blood losses replaced, certainly before ten percent of blood volume has been exceeded. This is particularly true in infants who are unable to maintain adequate arterial oxygen tensions with or without the use of supplemental inspired oxygen. At several weeks of age, when the clinical status of a preterm infant may have stabilized, transfusion may or may not be needed during the nadir of the anemia of prematurity. Infants who had been previously transfused or who had earlier received frequent simple transfusions should be able to tolerate lower levels of hemoglobin. Infants without compromised cardiopulmonary function and in whom no unusual metabolic needs exist are unlikely to be aided by transfusions when the hemoglobin concentration is greater than 10 to 11 g/dl. At lower levels of hemoglobin, simple calculations of "available oxygen" may be helpful when it is difficult to determine whether clinical signs and symptoms of anemia exist. Such signs and symptoms may include poor feeding, dyspnea, tachycardia, tachypnea, diminished activity, and pallor. Apnea has not unequivocably been shown to improve following transfusion. Clearly, our current concepts regarding indications for transfusion, even when based upon known principles of physiology, still represent an art form that is less than completely scientific.(ABSTRACT TRUNCATED AT 400 WORDS)

Anemia, Neonatal↗

PubMedDate not supplied

Acute loss of vision during pregnancy due to a suprasellar mass.

A pregnant woman presented with headaches, bilateral decreased visual acuity, and a central scotoma with a superotemporal hemianopic defect in the right eye and a superotemporal hemianopic defect in the left eye, and bilateral temporal optic disk pallor. Neuroimaging revealed an intrasellar mass with suprasellar extension. Biopsy of the lesion revealed lymphocytic hypophysitis (LH). Treatment with steroids produced marked improvement in visual function. The clinical presentation of lymphocytic hypothysitis may mimic pituitary adenoma and the diagnosis should be suspected in any pregnant or postpartum patient with an intrasellar or suprasellar mass.

Acute Disease↗

PubMedDate not supplied

Ophthalmoscopic evaluation of the optic nerve head.

Optic nerve diseases, such as the glaucomas, lead to changes in the intrapapillary and parapapillary region of the optic nerve head. These changes can be described by the following variables: size and shape of the optic disk; size, shape, and pallor of the neuroretinal rim; size of the optic cup in relation to the area of the disk; configuration and depth of the optic cup; ratios of cup-to-disk diameter and cup-to-disk area; position of the exit of the central retinal vessel trunk on the lamina cribrosa surface; presence and location of splinter-shaped hemorrhages; occurrence, size, configuration, and location of parapapillary chorioretinal atrophy; diffuse and/or focal decrease of the diameter of the retinal arterioles; and visibility of the retinal nerve fiber layer (RNFL). These variables can be assessed semiquantitively by ophthalmoscopy without applying sophisticated techniques. For the early detection of glaucomatous optic nerve damage in ocular hypertensive eyes before the development of visual field loss, the most important variables are neuroretinal rim shape, optic cup size in relation to optic disk size, diffusely or segmentally decreased visibility of the RNFL, occurrence of localized RNFL defects, and presence of disk hemorrhages.

Anthropometry↗

PubMedDate not supplied

Not so slowly progressive visual loss.

A 51-year-old man presented with bilateral progressive visual loss during a 2-month period. Visual acuity was 20/60 in both eyes with bilateral constricted visual fields. Funduscopy revealed bilateral disk pallor and arteriolar attenuation. His vision declined rapidly during the next 2 weeks. Investigations showed a positive cerebrospinal fluid Venereal Disease Research Laboratory test (1:8). A diagnosis of neurosyphilis was made, and treatment was started with high-dose intravenous and intramuscular penicillin.

Antibodies, Bacterial↗

PubMed1999

A snake bite by the Burrowing Asp, Atractaspis engaddensis.

During routine milking of a group of Burrowing Asps Atractaspis engaddensis, one of the authors was bitten in the index finger by one fang, as is characteristic of bites by snakes of the genus. Local effects, oedema, erythema and numbness appeared within minutes, followed by systemic effects, including general weakness, sweating, pallor, fluctuations in the level of consciousness, vomiting and watery non-bloody diarrhoea. Gross oedema of the hand developed and extended up to the forearm. Two hours after admission to the hospital, blood pressure rose to 180/110, the ECG showed normal sinus rhythm and no signs of atrioventricular conduction block. An ECG obtained 24 h after the bite showed new T-wave inversions in leads V5 + 6, which gradually returned to baseline within several days. The local effects healed during the following weeks, but some discoloration and tenderness remained even 10 months after the bite. A maximal exercise (SPECT) study carried out five months after the bite was normal and a multigated radionuclear ventriculogram (MUGA) showed normal left-ventricular function. It may be assumed that the rise in blood pressure observed in this case reflects a systemic vasoconstrictive effect of the sarafotoxins, while the ST changes may have been caused by the direct effect of the toxins on the heart or indirectly by vasoconstriction of the coronary arteries. However, ischaemia secondary to a rise in blood pressure or to excitement could also explain the observed ECG-changes.

Adult↗

PubMed1996

Dapsone in rheumatoid arthritis.

Dapsone, a synthetic sulfone with chemical similarities to sulfapyridine, has been used for a number of years to treat leprosy and dermatitis herpetiformis. Recently, a number of prospective, randomized, double-blind trials have shown their success in the management of rheumatoid arthritis, with dapsone being superior to placebo and comparable to chloroquine and hydroxychloroquine. Its mode of anti-inflammatory actions in rheumatoid arthritis is not clearly understood, but modulation of neutrophil activity or inhibition of neutrophil inflammatory product formation or release appear to play a role. The major limiting side effect is hemolytic anemia, which may be mitigated through careful patient selection, conservative drug dosing, close monitoring, and possibly, concurrent administration of antioxidants or cytochrome P450 inhibitors. Methemoglobinemia is another common finding among patients receiving dapsone therapy, but rarely does it result in prominent symptoms other than transient pallor. Less common adverse events to dapsone include the idiosyncratic reactions of leukopenia and agranulocytosis, cutaneous eruptions, peripheral neuropathy, psychosis, toxic hepatitis, cholestatic jaundice, nephrotic syndrome, renal papillary necrosis, severe hypoalbuminemia without proteinuria, an infectious mononucleosis-like syndrome, and minor neurological and gastrointestinal complaints. In this report, two patients with advanced rheumatoid arthritis, who were safely and effectively treated with dapsone after failure with other second-line agents, are described and the literature is reviewed. We suggest that dapsone is an effective second-line agent in the treatment of rheumatoid arthritis.

Anti-Inflammatory Agents, Non-Steroidal↗

PubMedDate not supplied

Clinical predictors of bioterrorism-related inhalational anthrax.

Limitation of a bioterrorist anthrax attack will require rapid and accurate recognition of the earliest victims. To identify clinical characteristics of inhalational anthrax, we compared 47 historical cases (including 11 cases of bioterrorism-related anthrax) with 376 controls with community-acquired pneumonia or influenza-like illness. Nausea, vomiting, pallor or cyanosis, diaphoresis, altered mental status, and raised haematocrit were more frequently recorded in the inhalational anthrax cases than in either the community-acquired pneumonia or influenza-like illness controls. The most accurate predictor of anthrax was mediastinal widening or pleural effusion on a chest radiograph. This finding was 100% sensitive (95% CI 84.6-100.0) for inhalational anthrax, 71.8% specific (64.8-78.1) compared with community-acquired pneumonia, and 95.6% specific (90.0-98.5) compared with influenza-like illness. Our findings represent preliminary efforts toward identifying clinical predictors of inhalational anthrax.

Aerosols↗

PubMed1989

Mass sociogenic illness by proxy: parentally reported epidemic in an elementary school.

In a cluster of illness reported among students at an elementary school parents mentioned many signs and symptoms including headache, pallor, dark circles under the eyes, nausea, and vomiting--which they attributed to exposure to recurrent leaks of natural gas at the school. It is likely that the parents spread among themselves the notion of toxic exposure at the school. A questionnaire revealed no spatial clustering, but increased reports of symptoms were related to intense media coverage. A thorough environmental and epidemiological investigation was negative, there being no evidence of a continuing gas leak or other potential causes. At a strictly biological level, the complaints in this reported "cluster" apparently represented the sporadic occurrence of common childhood illnesses. The possibility of an epidemic from toxic exposure at the school caused intense parental concern and led to a major public health problem. The established term "mass sociogenic illness" seems inapplicable here because complaints did not come principally from the students and the apparent epidemic illness was not transmitted among them. The term "mass sociogenic illness by proxy" is proposed to describe this incident, in which transmission in one group (the parents) resulted in reports of an epidemic in another group (students).

Absenteeism↗

PubMedDate not supplied

Dangling: a review of relevant physiology, research, and practice.

Although dangling is a common nursing intervention, little research has been conducted to test its effectiveness or to compare various dangling methods. By contrast, abundant information is available about orthostatic responses. In this article the authors explain the physiologic principles underlying orthostatic responses, focusing on blood volume distribution and the role of the mechanoreceptors, discuss typical and atypical responses to dangling and standing, describe clinical manifestations of orthostatic hypotension and syncope, present research-based practice guidelines, and, provide specific recommendations for future research. Because of the wide variability in heart rate and blood pressure responses to orthostasis, the authors stress the importance of signs and symptoms such as nausea, pallor, dizziness, visual dimming, and impaired consciousness in assessing orthostatic tolerance. Studying rituals such as dangling can advance nursing practice, improve patient outcomes, and move nursing to a research-based practice.

Adult↗

PubMed2004

French guidelines for the diagnosis and management of migraine in adults and children.

BACKGROUND: The French Recommendations for Clinical Practice: Diagnosis and Therapy of Migraine are guidelines concerning the overall management of patients with migraine, including diagnostic and therapeutic strategies and assessment of disability. OBJECTIVE: This article summarizes the guidelines as they apply to adults and children, and proposes future direction for steps toward optimal treatment of migraine in patients in France. METHODS: The recommendations were categorized into 3 levels of proof (A-C) according to the National Agency for Accreditation and Evaluation in Health (ANAES) methodology and were based on a professional consensus reached among members of the Working Group and the Guidelines Review Group of the ANAES. RESULTS: The International Headache Society diagnostic criteria for migraine should be used in routine clinical practice. Recommended agents for the treatment of migraine in adults include nonsteroidal anti-inflammatory drugs, acetylsalicylic acid (ASA) monotherapy or in combination with metoclopramide, acetaminophen monotherapy, triptans, ergotamine tartrate, and dihydroergotamine mesylate. Patients should use the medication as early as possible after the onset of migraine headache. For migraine prophylaxis in adults, the following can be used: propranolol, metoprolol, oxetorone, or amitriptyline as first-line treatment, and pizotifen, flunarizine, valproate sodium, or topiramate as second-line treatment. Migraine in children can be distinguished from that in adults by shorter duration (2-48 hours in children aged <15 years), more frequent bilateral localization, frequent predominant gastrointestinal disturbances, and frequent pallor hailing the onset of the attack. The following drugs are recommended in children and adolescents: ibuprofen in children aged >6 months, diclofenac in children weighing >16 kg, naproxen in children aged >6 years or weighing >25 kg, ASA alone or in combination with metoclopramide, acetaminophen alone or in combination with metoclopramide, and ergotamine tartrate in children aged >10 years. CONCLUSIONS: These guidelines are intended to help general practitioners to manage migraine patients according to the rules of evidence-based medicine.

Adolescent↗

PubMed2001

The prevalence of cupping in end-stage arteritic and nonarteritic anterior ischemic optic neuropathy.

OBJECTIVE: To evaluate the prevalence of cupping in arteritic anterior ischemic optic neuropathy (AAION) and nonarteritic anterior ischemic optic neuropathy (NAION). DESIGN: Retrospective, observational case series. PARTICIPANTS: Three hundred one patients. METHODS: Review of clinical records and color fundus photographs. MAIN OUTCOME MEASURES: Photographic and clinical interpretation of optic nerve appearance. RESULTS: Ninety-two patients with AAION and 102 with NAION were included in the study. Disc photographs of 42 patients (48%) with AAION and 32 patients (31%) with NAION were available for reassessment. These were presented in a masked fashion along with a random sample of 27 disc pairs considered to be 'normal' and 27 disc pairs with 'established glaucoma' according to two examiners. Cupping was present in 92% of eyes with AAION secondary to giant cell arteritis and in 2% of eyes with NAION (kappa = 0.96; P < 0.001). CONCLUSIONS: The end-stage optic disc appearance in AAION secondary to giant cell arteritis is cupping, whereas segmental or diffuse pallor without cupping is the typical disc appearance after NAION.

Aged↗