Two siblings with unilateral renal agenesis

Family with two consecutive brothers with unilateral aplasia of the kidney are reported. Role of genetic inheritance and defects in embryogenesis of familial renal aplasia are discussed.     

Indigenous automatic peritoneal dialysis unit

Automatic peritoneal dialysis unit has been developed and fabricated indigenously from multiple electronic and electro-magnetic components available in India. Flow of fluid in and out of the peritoneal cavity is controlled automatically. Dialysis fluid of each cycle is weighed mechanically. If flow of fluid is inadequate the unit gives both audio and visual alarm for immediate attention. Number of cycles and total return of the fluid can be observed at any time. Risk of infection has been minimised as there is no need to change the dialysis fluid at each cycle. There is no need to autoclave the unit as the dialysis fluid does not come in direct contact with the unit. Running cost of the unit is only the cost of dialysis fluid. With easy availability of this unit the peritoneal dialysis facilities can be made avialable in each hospital and even at home in children with chronic ronal failure. This will improve the health care of children with renal failure. The approximate cost of the unit will be Rs. 10,000 only.     

Bispectral index monitoring: a comparison between normal children and children with quadriplegic cerebral palsy

We performed this study to compare the correlation of bispectral index (BIS) values with different sevoflurane concentrations between normal children and those with quadriplegic cerebral palsy with mental retardation (CPMR). Twenty children with CPMR (Group I) and 21 normal children (Group II) between 2 and 14 yr of age were studied. Anesthesia was induced and maintained with sevoflurane and 66% N(2)O/O(2). Bispectral values were recorded on an Aspect Medical Systems (Natick, MA) monitor, and sevoflurane concentrations were measured with an Ohmeda (Hanover, MA) inhaled anesthetic monitor. The BIS values were recorded after midazolam premedication; after the induction of anesthesia; at end-tidal sevoflurane concentrations of 1%, 3%, and again at 1%; and after emergence from the anesthetic. Both groups were similar in age and sex distribution, but children in Group I weighed less than those in Group II (P < 0.05). The BIS values were significantly lower in Group I compared with Group II after sedation, at 1% sevoflurane concentrations, and after emergence. No difference was observed between the two groups at anesthesia induction (8%) and at 3% sevoflurane concentration. We conclude that, in children with CPMR, BIS values exhibit a pattern of change similar to that observed in normal children. However, absolute BIS values obtained in such children are lower than those in normal children while awake and at different sevoflurane concentrations. IMPLICATIONS: We compared bispectral (BIS) values with different sevoflurane concentrations between normal children and children with cerebral palsy. We observed that, in children with cerebral palsy, BIS values exhibited a similar pattern of change as is observed in normal children. However, absolute BIS values obtained in such children are lower than those in normal children while awake and at different sevoflurane concentrations.     

Larsen syndrome and its anaesthetic considerations

Larsen syndrome is a complex syndrome with genetic heterogeneity, and with both autosomal dominant and autosomal recessive patterns of inheritance. It is characterized by congenital dislocation of joints, flat faces and complicated by issues relating to respiratory, cardiac, musculoskeletal and central nervous systems. This report describes the anaesthetic management of two patients with Larsen syndrome. The first case is a 4-year-old patient who had cervical cord compression secondary to cervical instability and who was scheduled for anterior corpectomy with fusion of cervical vertebrae and placement of halo frame. This patient had transient loss of evoked potentials during positioning and a stormy postoperative course requiring reintubation and a prolonged stay in the intensive care unit. The second case is a 22-month-old child who was scheduled for a repeat posterior cervical spinal fusion due to failure of her initial fusion procedure. This patient had an uneventful perioperative course. Relevant anaesthetic issues in patients with Larsen syndrome are discussed.