Living Donor Kidney Transplantation in a Patient With Epidermolysis Bullosa: A Case Report.

Severe recessive dystrophic epidermolysis bullosa is a very rare inherited disease with excessive blisters forming starting at birth. Surgical intervention in this population creates a challenge: preventing formation of new lesions while managing previously scarred tissues. We present a case of a 27-year-old patient with end-stage renal disease caused by rapidly progressive IgA nephropathy. Living donor kidney transplantation was performed under local, spinal and epidural anesthesia. Living kidney transplantation in epidermolysis bullosa patients with end-stage renal disease should not be a contraindication for transplantation and should be considered as a viable and feasible option after careful preparation.

[Donor nephrectomy: less fatigue and better quality of life following laparoscopic kidney removal compared with an open procedure by mini-incision: blind randomised study]. / Donornefrectomie: minder vermoeidheid en meer kwaliteit van leven na een laparoscopische uitname dan na een open ingreep met een mini-incisie: geblindeerd, gerandomiseerd onderzoek.

OBJECTIVE: Determining possible differences in living donor nephrectomy procedures: laparoscopy against mini-incision concerning discomfort to the donor and the maintenance of good graft function. DESIGN: Blind randomized study. METHOD: In two university medical centres, one hundred living kidney donors were randomly assigned to either total laparoscopic donor nephrectomy or mini-incision muscle-splitting open donor nephrectomy. Primary outcome was physical fatigue measured with the 'Multidimensional Fatigue Inventory' (MFI-20) during one-year follow-up. Secondary outcomes were physical function measured with the 'Short form-36' questionnaire, postoperative hospital stay, amount of pain, operating times and graft and patient survival. RESULTS: Donors who underwent laparoscopy experienced less fatigue (difference: -1.3; 95% CI: -2.4 - (-0.1)) and physical function was better (difference: 6.2; 95% CI: 2.0-10.3) during one-year follow-up. Those donors who underwent laparoscopy required less morphine (16 mg versus 25 mg; p = 0.005) and the duration of hospital stay was shorter (3 versus 4 days; p = 0.003). The laparoscopic procedure resulted in a longer operation time (221 versus 164 min; p < 0.001) a longer first warm ischaemia time (6 versus 3 min; p < 0.001) and less blood loss (100 versus 240 ml; p < 0.001). Recipient renal function and one-year graft survival rates did not differ. The number of preoperative and postoperative complications did not differ significantly between both surgery techniques. Conversions did not occur. CONCLUSION: Donor nephrectomy through laparoscopy led to less fatigue and a better quality of life compared with the open procedure. The safety factors for donors and recipients were comparable for both techniques. Laparoscopic donor nephrectomy is therefore the better surgical choice for kidney donor programmes with living donors.

Effect of intraabdominal pressure elevation and positioning on hemodynamic responses during carbon dioxide pneumoperitoneum for laparoscopic donor nephrectomy: a prospective controlled clinical study.

BACKGROUND: Carbon dioxide (CO2) pneumoperitoneum (PP) increases mean arterial blood pressure (MAP) and systemic vascular resistance (SVR) but decreases stroke volume (SV) and cardiac output (CO). This study evaluated the hemodynamic effects of elevated intraabdominal pressure (IAP) occurring during laparoscopic donor nephrectomy (LDN). METHODS: Twenty-two patients undergoing LDN were investigated and hemodynamic parameters, P(v)CO2) (carbon dioxide partial pressure), and VCO2 (carbon dioxide production) were monitored during the procedure. Before and after PP, IAP was raised from 12 to 20 mmHg and the hemodynamic effects were measured every 30 s. RESULTS: During IAP of 12 mmHg and stable serum CO2, there was no change in SV compared to preinsufflation levels. When IAP was elevated from 12 to 20 mmHg, SV initially decreased (p < 05), followed by an increase in MAP and SVR (p < 0.05). CONCLUSION: This study shows that with the fluid and ventilation protocol used, PP has no significant effect on SV at an IAP of 12 mmHg, whereas increasing IAP to 20 mmHg does. In this study, the hemodynamic effects induced by CO2 PP of 12 mmHg are not due to changes in serum CO2. Compression of the venous system during a PP of 20 mmHg reduces preload, with an subsequent increase in SVR.

Hearing loss related to zinc deficiency in rats.

Based on the observation that the zinc concentration in the cochlea of guinea pigs is very high, Shambaugh in North America has suggested that zinc plays an important role in hearing. Zinc deficiency is not infrequently present in growing children and elderly people, and thus might result in a hearing loss, which should be treated with zinc supplementation. The present experiment examined the effects of a zinc-deficient diet on hearing in 7-week-old rats. The auditory brainstem response threshold was measured in four rats before, during and after a zinc-deficient diet. Concentrations of zinc in the brain and in several other organs were measured, and the cochleas were examined microscopically. No hearing loss or morphological change in the cochlea of these animals was detected, although a clear zinc-deficient status was reached.

Dietary zinc deficiency has no effect on auditory brainstem responses in the rat.

Zinc has been shown to effect--in vitro--a number of processes associated with neurotransmission. We have tested whether the rate of impulse conduction--in vivo--as measured from the latencies of auditory brainstem responses (ABR), is influenced by dietary zinc deficiency in the rat. Dietary zinc deficiency for up to 26 wk had no effect on the wave I-IV interval compared to zinc-adequate fed animals. The results are discussed in relation to the observed constancy of brain overall and extracellular fluid zinc concentrations under conditions of dietary zinc deficiency.

Correlations between cis-platinum dosage and toxicity in a guinea pig model.

A guinea pig model was used to study correlations between cis-platinum dosage and level of hearing loss, hair cell loss in the cochlea. Other parameters measured included weight loss and serum urea, creatinine, and cis-platinum levels. The damage to the inner ear expressed as hearing loss and hair cell loss demonstrated a highly positive correlation with the dosage of cis-platinum, and only a moderately positive correlation with the cis-platinum serum levels. Weight loss correlated well with hearing loss.

Development of cis-platinum-induced hearing loss in guinea pigs.

The resultant hearing loss can be variable if cis-dichlorodiammineplatinum is given to guinea pigs. In order to find out more about these differences, we used brainstem audiometry to study the start and development of hearing loss over time for several frequencies. Our results confirmed previous observations that hearing loss starts at the higher frequencies but can also occur at lower frequencies. Furthermore, there were great differences in the start and the rate of the increase of hearing loss found in the individual animals. The individual differences in susceptibility can not be explained by one factor alone, but by the combination of three factors, namely deviation point, slope and length of survival.