Search results for "Maldevelopment"

Intra- and Interhemispheric Electroencephalogram Coherence in Siblings Discordant for Schizophrenia and Healthy Volunteers

1997

Former studies had pointed to an increased electroencephalogram (EEG) coherence in schizophrenics, but it remained unsolved whether this deviation represents the premorbid state or is only a consequence of the current or previous schizophrenic episodes. To clarify this question, we tested the hypothesis that subjects at elevated risk also reveal higher coherences compared to healthy controls. For that, intra- and interhemispheric EEG coherences were investigated in untreated schizophrenics, their healthy siblings, and healthy controls. Differences were only found regarding the intrahemispheric coherences. Both in schizophrenics and, even though to a lesser degree, in their siblings signific…

Studies of malformation syndromes of man XIB: the cerebro-hepato-renal syndrome of zellweger: Comparative pathology

1976

A study of 11 autopsied cases of the cerebro-hepato-renal syndrome of Zellweger (ZS) is reported. All cases had severe, persistent congenital hypotonia, hepatic lobular disarray, renal cortical cysts and pulmonary hypoplasia. Many had cardiovascular malformations, hepatomegaly, cerebral cortical gyral maldevelopment and pancreatic islet hyperplasia. Additional, less frequent findings are delineated. Results of iron content studies of hepatic and renal tissues are related to age of survival and possible development of fibrosis.

The Fate of Nephrons in Congenital Obstructive Nephropathy: Adult Recovery is Limited by Nephron Number Despite Early Release of Obstruction

2015

Urinary tract obstruction and reduced nephron number often occur together as a result of maldevelopment of the kidneys and the urinary tract. We determined the role of nephron number on adaptation of the remaining nephrons of mice subjected to neonatal partial unilateral ureteral obstruction followed through adulthood.Wild-type and Os/+ mice (the latter with 50% fewer nephrons) underwent sham operation or partial unilateral ureteral obstruction in the first 2 days of life. Additional mice underwent release of unilateral ureteral obstruction at 7 days. All kidneys were harvested at 3 weeks (weaning) or 6 weeks (adulthood). Glomerular number and area, glomerulotubular junction integrity, prox…

Addressing the Pandemic in the Philippines Necessitates a New Economic Paradigm

2020

Pathomorphology of hereditary sensory neuropathies

1995

Currently, the hereditary sensory neuropathies (HSN) — because of the involvement of the autonomic system recently called HSAN — comprise types I–V predicated on clinical differences. This classification of HSAN I–V seems to be uncontested, at the present time. Morphologically, individual forms I–V only differ in the non-specific loss or lack of myelinated and unmyelinated nerves in varying degrees in that in HSAN II large myelinated axons are most affected, in HSAN IV unmyelinated axons are almost absent; but each HSAN is considered an axonal type of neuropathy. Early onset, slow or no progression of the neuropathic process, and little or no evidence of ongoing degeneration suggest maldeve…

Congenital nephrosis, mesangial sclerosis, and distinct eye abnormalities with microcoria: An autosomal recessive syndrome

2004

We observed the occurrence of congenital nephrotic syndrome (CNS) and distinct ocular anomalies in two unrelated families. Eleven children from both families presented with a similar course of renal disease starting with nephrotic syndrome and renal failure prenatally or immediately after birth that resulted in death before the age of 2 months. Kidney histopathology showed diffuse mesangial sclerosis (DMS). Clinically obvious eye abnormalities were recognized in six of the eight patients in whom sufficient clinical data were available. Ocular anomalies included enlarged or large appearing corneae in some cases suggesting buphthalmos, and extremely narrow, nonreactive pupils (microcoria). Pa…

Morphology of placental villi after premature delivery and its clinical relevance

1986

Based on a new concept of maturation of the placental villous tree and its disorders (synchronous and asynchronous immaturity, asynchronous maturity, hyperpermaturity, and terminal villi deficiency) we studied the possible effect of the placental villous tree on the premature onset of labour. In mature normal neonates irregular and asynchronous villous patterns were found in 50% of cases. In prematurely delivered neonates, only 33% of the corresponding placentas show synchronous immature villous patterns. Uterine bleeding in the first trimester was associated with a 42% of incidence of premature maturation of the villous tree. These findings strengthen the idea that hormonal imbalance in ea…