The Low‐weight Groups and Haemodialysis

Ahola, T; Björkman, Helinä; Mäkelä, P H; Pasila, M; Vilska, J.; Hallman, N

doi:10.1111/j.1651-2227.1972.tb15895.x

Cited by 13 publications

(4 citation statements)

References 6 publications

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“…The early phase of UTS Type-II, with severe hypotonia, no ambulation, truncal ataxia, severe psychomotor retardation, no speech, and a marked cerebellar hypoplasia, may be related to the long-known DES (Hagberg et al, 1972) and its clinical synonym Cayman ataxia (Brown et al, 1984; Nystuen et al, 1996). In contrast to these non-progressive cerebellar ataxias, in the UTS Type-II cases, the severe muscular hypotonia gradually disappeared and was replaced by strong muscles appropriate for walking, at least on all four extremities.…”

Section: Discussionmentioning

confidence: 99%

Two families with quadrupedalism, mental retardation, no speech, and infantile hypotonia (Uner Tan Syndrome Type-II); a novel theory for the evolutionary emergence of human bipedalism

Tan

2014

Front. Neurosci.

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Two consanguineous families with Uner Tan Syndrome (UTS) were analyzed in relation to self-organizing processes in complex systems, and the evolutionary emergence of human bipedalism. The cases had the key symptoms of previously reported cases of UTS, such as quadrupedalism, mental retardation, and dysarthric or no speech, but the new cases also exhibited infantile hypotonia and are designated UTS Type-II. There were 10 siblings in Branch I and 12 siblings in Branch II. Of these, there were seven cases exhibiting habitual quadrupedal locomotion (QL): four deceased and three living. The infantile hypotonia in the surviving cases gradually disappeared over a period of years, so that they could sit by about 10 years, crawl on hands and knees by about 12 years. They began walking on all fours around 14 years, habitually using QL. Neurological examinations showed normal tonus in their arms and legs, no Babinski sign, brisk tendon reflexes especially in the legs, and mild tremor. The patients could not walk in a straight line, but (except in one case) could stand up and maintain upright posture with truncal ataxia. Cerebello-vermial hypoplasia and mild gyral simplification were noted in their MRIs. The results of the genetic analysis were inconclusive: no genetic code could be identified as the triggering factor for the syndrome in these families. Instead, the extremely low socio-economic status of the patients was thought to play a role in the emergence of UTS, possibly by epigenetically changing the brain structure and function, with a consequent selection of ancestral neural networks for QL during locomotor development. It was suggested that UTS may be regarded as one of the unpredictable outcomes of self-organization within a complex system. It was also noted that the prominent feature of this syndrome, the diagonal-sequence habitual QL, generated an interference between ipsilateral hands and feet, as in non-human primates. It was suggested that this may have been the triggering factor for the attractor state “bipedal locomotion” (BL), which had visual and manual benefits for our ape-like ancestors, and therefore enhancing their chances for survival, with consequent developments in the psychomotor domain of humans. This was put forward as a novel theory of the evolution of BL in human beings.

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Section: Discussionmentioning

confidence: 99%

Two families with quadrupedalism, mental retardation, no speech, and infantile hypotonia (Uner Tan Syndrome Type-II); a novel theory for the evolutionary emergence of human bipedalism

Tan

2014

Front. Neurosci.

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“…Whereas the overall mortality of infants on dialysis may be as high as 16%, there are no comparable figures available for patients on HD, due to inadequate numbers [3]. Data available concerning HD in infants are restricted to infants with acute renal failure [4] or are based on publications from the 1970s [5,6]. Since then, volumetrically controlled dialysis machines have been introduced, vascular access, nutritional practice, and medications have changed.…”

Section: Introductionmentioning

confidence: 99%

Hemodialysis for end-stage renal disease in children weighing less than 10 kg

Al-Hermi

Alsaran

Secker

et al. 1999

Pediatric Nephrology

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Hemodialysis (HD) of infants with end-stage renal disease (ESRD) is technically difficult and labor intensive, although there are few data in the literature to document the outcomes of this treatment. We retrospectively reviewed all patients with ESRD who received HD between 1983 and 1997 who weighed <10 kg at the beginning of HD. A total of ten patients aged 2-27 months, weighing 3.5-9.5 kg, were identified. All patients were dialyzed through a central venous line; three had a failed sapheno-femoral loop and one a failed brachial shunt. Line clot was observed in nine and line sepsis in six patients. Subclavian vein stenosis was documented in one patient following removal of a clotted subclavian line. The mean urea reduction ratios calculated during the 1st and 3rd month of HD were only 54% and 49%, respectively. Anemia was a frequent problem, despite the use of erythropoietin in seven of the infants. Outcomes included: successful renal transplant in four, switch back to peritoneal dialysis in two, improved renal function and dialysis discontinuation in one, and death after withdrawal of treatment in three patients. All three patients who died were <5 months of age, weighed <5 kg, and were anuric; two of the three had congenital nephrotic syndrome. In conclusion, successful HD is possible in small children with ESRD, but morbidity is substantial and mortality is high.

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“…Several reports on hemodialysis (HD) in infants and small children were published during the 1970s, but during the last decade continuous peritoneal dialysis (PD) has become the modality of choice in the majority of small children with end-stage renal disease (ESRD) [1,2]. However, between 1986 and 1988 in Europe and the United States, in 10%-30% of all children with ESRD less than 2 years of age, HD was the first method of renal replacement therapy [3,4].…”

Section: Introductionmentioning

confidence: 99%