Familial achalasia associated with adrenocortical insufficiency, alacrima, and neurological abnormalities

We report on two brothers with achalasia, adrenocortical insufficiency, alacrima, short stature, microcephaly, ataxia, optic atrophy, and developmental delay. The parents and three sibs are unaffected. Achalasia, adrenocortical insufficiency, and alacrima comprise a recently characterized familial multisystem disorder of unknown cause. Achalasia has also been described in association with microcephaly and mental retardation in one family and ataxia, optic atrophy, and mental retardation in another. The above reports and these sibs may represent variants of a single pleiotropic recessive gene. We suggest that abnormalities of the central nervous system are a manifestation of the achalasia, adrenocortical insufficiency, alacrima syndrome.     

EDTA Registry centre survey, 1986. Report from the European Dialysis and Transplant Association Registry

This paper summarises the information given on the 1986 EDTA Registry centre questionnaire which was returned by 82% of the 2,065 known dialysis and transplant centres in 33 European countries. Information is given on the number of patients alive on haemodialysis according to the type of dialysis facilities available where the patient was receiving dialysis and the number of patients receiving special types of dialysis. The centre questionnaire also included questions on testing for HIV infection, serological evidence or symptoms of AIDS and the diagnosis of hepatitis B in patients and staff. The data given in response to these questions are presented together with data on the involvement of dietitians and social workers in the treatment of patients with end stage renal failure. Finally, information on transplant activity in Europe and the treatment policies of transplanting centres is provided.     

Daily SOAP

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Diseases involving the heart and kidney in children and adults]

An association of renal and cardiac manifestations can be observed in a variety of diseases. The pathogenetic factors may include, firstly, a disturbance of organ development due to chromosomal aberration or an underlying syndrome or, secondly, infectious, immunologic and metabolic factors. In addition, primarily isolated diseases of the heart or the kidney may lead to damage of the previously unaffected organ. The present article gives a review on renal diseases associated with heart diseases in children and adults.     

Pathophysiological and clinical aspects of the CO2 pneumoperitoneum (CO2-PP)

Experimental studies demonstrated a severe cardiac load of the CO₂ pneumoperitoneum caused by an accelerated after- and a decreased preload. Patients displaying cardiovascular risks are therefore often rejected from laparoscopic surgery. Hence, the pathophysiological changes and the intraoperative risk of the CO₂ pneumoperitoneum in high-risk cardiopulmonary patients (NYHA II–III, n= 15) undergoing laparoscopic cholecystectomy are described. The changes in cardiac after- and preload seem to be due to the elevated intraabdominal pressure rather than transperitoneally resorbed CO₂ and are reversible by desufflation. In one patient conversion to open operation had to be performed because of a severe drop in cardiac output and right ventricle ejection fraction. Mixed oxygen saturation was predicting intraoperative worsening in this case. The described pathophysiological changes may seem to be well tolerated even in high-risk cardiac patients. Monitoring of hemodynamics should include an arterial catheter line and blood gas analyses. Pharmacologic interventions or pressureless laparoscopic procedures might not be necessary as long as laparoscopic cholecystectomy is performed. Received: 13 December 1996/Accepted: 8 January 1997     

Sind „Linkshänder” bessere Lokalanästhetika? Zur Relevanz der Stereoisomerie in der klinischen Praxis am Beispiel neuer Lokalanästhetika

S-Ropivacaine is a new, long-acting amide local anaesthetic. It is the first local anaesthetic to be on the market as a single isomer. Its pharmacodynamic and pharmacokinetic profile is similar to that of bupivacaine. In vitro and in vivo experiments have shown ropivacaine to be less cardiotoxic than bupivacaine. When given epidurally, both local anaesthetics are equally effective in producing sensory block, but motor block seems to be less pronounced in the case of ropivacaine. This pharmacodynamic profile suggests that ropivacaine has a greater margin of safety and should be a valuable candidate in applications where motor blockade is to be avoided, e.g. for postoperative epidural analgesia and for epidural analgesia in obstetrics.     

Comparative dose-response studies of organophosphorus ester-induced delayed neuropathy in rats and hens administered mipafox.

A single injection of mipafox was administered to both Long-Evans hooded rats and White Leghorn hens in dosages which inhibited the activity of brain neurotoxic esterase 30-50%, 60-80%, or greater than 80% four hr after intoxication. All animals were monitored for clinical evidence of organophosphorus induced delayed neuropathy for 21 days, euthanatized, and regions of the nervous system were histologically evaluated. Only hens manifested clinical signs of neuropathy; however, light and electron microscopic lesions were present in the nervous systems of both species. In rats, these lesions were well developed in only the highest dosage group and confined to the rostral level of the fasciculus gracilis in the medulla oblongata. Swollen axons containing a single vacuole filled with flocculent material were the most prominent lesion in rats. Hens manifested more extensive and varied fiber breakdown in multiple spinal cord tracts, with the intensity of degeneration increasing with increasing dosages of mipafox. Both marked Wallerian-like degeneration and swollen axons filled with aggregates of cellular debris were observed in the nervous systems of hens. This study indicates that both rats and hens are susceptible to OPIDN. However, there are qualitative and quantitative differences in both clinical manifestations and histologic appearances between the two species.     

Frühgeburt und Grenzen

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