Nutrition Management of Patients with Epidermolysis Bullosa

Epiderrnolysis bullosa (EB), a heterogeneous group of rare, inherited disorders, is manifested by recurrent blistering of the skin induced by the slightest trauma. Little information exists regarding the nutrition management of patients with EB. This study presents information on growth, identifies potential nutrition problems, and provides guidelines for nutrition management of persons with EB. Eighty patients attending a dermatology clinic for EB patients are described. Severity of disease ranged from mild blistering of the knees, elbows, and feet to extensive blistering and scarring of the skin and entire gastrointestinal tract. Of the 18 children with EB simplex, which is a mild form of the disease, 4 (22%) were at nutritional risk. None of the 13 adults with EB simplex were underweight and 8 (62%) were overweight. Of the patients with the more severe forms of EB, 27 of the 35 (77%) children with dystrophic EB and 4 of the 7 (57%) children with junctional EB were at risk for malnutrition. Of the 7 adults with dystrophic EB, 6 (86%) were underweight. Common nutrition problems included protein-energy malnutrition, chewing and swallowing problems, constipation, anemia, and vitamin/mineral deficiencies. When nutrition care protocols address these problems, growth, development, and nutritional status can improve. For those with severe nutrition problems, gastrostomy feeding or similar nutrition therapies should be considered. J Am Diet Assoc. 1995; 95:575-579.     

Endoscopic transthoracic sympathectomy for idiopathic upper limb hyperhidrosis

A 5-Year experience of 51 endoscopic transthoracic dorsal sympathectomies for idiopathic palmar hyperhidrosis in 26 patients is presented. Fifty-two percent complained of excessive sweating over their hands, 28% of axillary sweating and 20% over both areas, with a mean duration of 10 years. The second, third and fourth thoracic ganglia and their interconnecting fibres on the affected side were ablated using diathermy cautery. Over a mean follow up time of 26 months, this procedure was successful in curing or improving intractable sweating in 92%. However, axillary sweating was less well controlled than in the palms with 20% of patients describing residual wetness in the axilla. Compensatory sweating (75%) and gustatory sweating (48%) were the commonest side effects; despite this, most patients were satisfied with the functional and cosmetic outcome. Other complications included a temporary Horner's syndrome in one patient, a pneumothorax in the immediate post-operative period in another and a unilateral non-infective reactionary pleural effusion in a third. Two patients developed recurrence of palmar hyperhidrosis within 6 months of surgery. One has been successfully treated by re-operation on the affected side. All patients complained of mild to moderate interscapular chest pain which was easily controlled by non-steroidal anti-inflammatory agents, and resolved within 7–10 days post-operatively. The technique of endoscope transthoracic sympathectomy is effective, relatively simple to perform and usually requires only an overnight stay. It is recommended as the surgical treatment of choice for upper limb hyperhidrosis unresponsive to conservative measures.     

Family system test (FAST): Are parents' and children's family constructs either different or similar,or both?

Perceptions of family cohesion and hierarchy structures were assessed by theFamily System Test (FAST), a clinically-derived figure placement technique. Parents (N=140) and their preadolescent offspring (N=70) completed typical and conflict representations in individual as well as group settings. Typical representations were characterized by balanced family structures (i.e. cohesive and moderately hierarchical) and those displaying conflict situations showed predominantly unbalanced patterns. FAST portrayals were related to respondent (mother vs. father vs. child). Fathers represented typical family relations as balanced more often than mothers. Regarding conflict representations, children were more likely than fathers to portray the family as unbalanced. However, analyses of representations of the same family (i.e. intra-family comparisons) indicated that all respondents differed in their perceptions and, that fathers' typical portrayals showed most often the same structure as those done by the family members as a group.     

Modified Kalish osteotomy: A simple approach to minimizing proximal dorsal stress risers

Proximal dorsal stress risers are a potential complication to the Kalish osteotomy. The authors describe a modification to this osteotomy that is simple and effective. Since performing the wing-clip modification, there have been no observed stress risers.     

Identification of Alcohol Abuse and Alcoholism with Biological Parameters

The prevalence and incidence of heavy alcohol consumption are major problems which have been increasing in many countries in recent years. It is crucial for physicians to consistently identify early drinking problems as well as the various end disease states in order to minimize suffering and maximize recovery. This paper reviews the evolutionary development of clinical tools for detection of alcohol abuse. The focus is primarily on clinical/biochemical indicators of alcohol abuse, emphasizing but not limited to changes in hematological characteristics, liver enzyme activity, lipids, immune function factors, hormones, neurological factors, and some physically based tests. Use of test combinations and sophisticated statistical analysis of pattern changes in test batteries evidence increased diagnostic efficiency.     

Family History of Alcoholism Does Not Predict Neuropsychological Performance in Alcoholics

We examined the relationship of history of alcoholism in first-degree relatives to neuropsychological performance of alcoholics abstinent from several weeks to several years. Eighty-four men were assigned to four groups based on "strength" of family history of alcoholism. The groups were: (1) "strong history," a parent plus another first-degree relative positive; (2) "moderate," parent only positive; (3) "weak," nonparent first-degree relative only positive; and (4) "negative," no first-degree relative positive. There were no significant between-group differences in NP performance. In other analyses there were no NP differences between alcoholics classified positive or negative purely on basis of paternal alcoholism, and no differences between subjects who had multigenerational versus unigenerational versus negative familial histories of alcoholism. It is concluded that genetic loading for alcoholism does not significantly affect the NP status of abstinent alcoholic groups equated for education, drinking history, and medical risk.     

A Retrospective Review of the Use and Safety of Droperidol in a Large, High-risk, Inner-city Emergency Department Patient Population

Droperidol (DROP) is used in the emergency department (ED) for sedation, analgesia, and its antiemetic effect. Its ED safety profile has not yet been reported in patients (pts). OBJECTIVES: To document the use of DROP in high-risk pts (those with head injury, alcohol or cocaine intoxication, and/or remote or recent seizures), and to determine the number of serious and minor adverse events (AEs)-seizures, hypotension, extrapyramidal side effects (EPSEs)-after DROP. METHODS: The ED database (EmSTAT) was queried to determine who received intramuscular or intravenous DROP in the ED in 1998; further chart review was done if the patient was considered high risk for or had experienced an AE. Multiple regression analysis using a random-effects model determined the significance of each variable in the occurrence of AEs. RESULTS: 2,468 patients (aged 20 months to 98 years; 112 < or =17 years; 141 > or =66 years) received DROP for agitation (n = 1,357), pain (1,135), anxiety (99), vomiting (173), or other reasons (50). There were 945 pts considered high risk; 933 charts were reviewed (DROP mean dose 4.1 +/- 2.0 mg); of these, 50 patient visits did not meet the criteria for high risk. There were 622 pts with head trauma (401 with alcohol use), including 47 with computed tomography (CT) scans positive for brain injury, 64 with cocaine use, and 197 with recent or remote seizures (137 with alcohol use). Minor AEs such as transient hypotension occurred in 96 pts after DROP (73 with alcohol use); 20 received intravenous fluids, while an additional 28 pts (8 with alcohol use) received rescue medications for EPSEs. Six possible serious AEs occurred in pts with serious comorbidities; 2 cases of respiratory depression, 3 post-DROP seizures, and 1 cardiac arrest (resuscitated) 11 hours after DROP in a cocaine-intoxicated pt (normal QT interval). There was no significant difference among high-risk groups in the occurrence of AEs. CONCLUSIONS: The vast majority of pts who received DROP in the ED did not experience an AE. A few serious AEs were noted following DROP in patients with serious comorbidities; it is not clear that DROP was causative.