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Biomedical subjects

W D Grover

Publications and source records attributed to W D Grover.

At least 37 records · Page 2Linked to original sources
PubMed1981

Encephalopathy in infants and children with chronic renal disease.

The examination of five pediatric patients with encephalopathy secondary to chronic renal failure has indicated a stereotyped sequence of neurologic signs and symptoms including ataxia, loss of motor abilities, myoclonus, seizures, dementia, and bulbar dysfunction. Both the patients with CNS dysfunction and a control group selected for a similar degree of renal failure had increased levels of serum phosphate, alkaline phosphatase, and parathyroid hormone. Serial EEGs in the affected group revealed progressive slowing and an increase in paroxysmal features. No specific neuropathologic findings were noted in one patient.

Brain Diseases↗
PubMed1980

Serum copper concentrations in sick and well preterm infants.

In order to define the range of serum copper concentrations in preterm infants and to determine the effect of growth upon these values, serial serum copper concentrations were measured in 26 preterm infants over their first six weeks of life. Fourteen healthy, growing preterm infants (Group I) had mean serum copper concentrations below 32 microgram/dl throughout the study. Clinical and hematologic signs of copper deficiency which responded promptly to the oral administration of copper sulfate were noted in five of these 14 infants. Twelve ill preterm infants (Group II-A), who received parenteral nutrition without supplemental copper and had slow rates of growth, had mean serum copper concentrations above 50 micrograms/dl after the first week of life. Seven surviving from Group II-A (Group II-B) had a decrease in mean serum copper concentrations to values similar to those in Group I after two weeks of oral feedings and resumption of normal growth. Our findings suggest that preterm infants who have normal growth while receiving oral feedings are at significant risk for developing copper deficiency.

Blood Transfusion↗
PubMed1979

Clinical and biochemical aspects of trichopoliodystrophy.

The clinical and biochemical evaluation of 6 patients with trichopoliodystrophy indicates that the disease process can begin in utero and is related to a selective abnormality in copper metabolism. Examination of 2 infants on the first day of life revealed abnormal neurological signs, a characteristic hair abnormality, and elevated levels of copper and ceruloplasmin. Decreased hepatic copper levels and increased urinary copper excretion were documented during the first week. The 2 neonates demonstrated a progressive decrease in blood copper levels in the first month of life. Four infants identified at ages 2 to 11 months had low values for blood copper and ceruloplasmin. All infants had progressive neurological dysfunction, and 4 of the 6 died at ages ranging from 2 1/2 months to 5 1/2 years. Parenteral copper therapy achieved normal blood and hepatic copper levels in 1 patient, but the copper values in the cerebral cortex and white matter were significantly decreased compared to control specimens.

Amniotic Fluid↗
PubMed1978

Clinical variation in 2 related children with neuronopathic Gaucher disease.

The clinical features in 2 second cousins with neuronopathic Gaucher disease include slowly progressive ataxia, spasticity, myoclonus, and seizures with relative preservation of intellectual function. Organomegaly was noted only in Patient 1. Both patients had diffuse slowing with paroxysmal features in electroencephalograms and a deficiency of beta-glucosidase activity in leukocytes and skin fibroblast cultures. The parents of Patient 1 and the related father of Patient 2 had levels of beta-glucosidase activity consistent with the carrier state for Gaucher disease. The value of beta-glucosidase activity in the mother of Patient 2 suggests a different mutation, the result being a defective enzyme component not detectable by measuring total activity.

Child↗
PubMed1977

Globoid cells, glial nodules, and peculiar fibrillary changes in the cerebro-hepato-renal syndrome of Zellweger.

In addition to a distinct malformation (pachymicrogyria, heterotaxic lamination of the cerebellar cortex, olivary dysplasia), unusual degenerative changes were found in the nervous system of 2 unrelated babies with the Zellweger syndrome. Cerebral clefts were present in 1 case. In both infants there was neuron loss and accumulation of glial nodules and globoid cells in the gray matter as well as degeneration of the white matter. There was fatty change in astrocytes and diffuse gliosis. Neurons in the column of Clarke and the lateral cuneate nucleus showed peculiar fibrillary changes. Cytoplasmic inclusion bodies were seen in the spinal ganglia. Swelling of cortical astrocytes was remarkable in the older infant. The combination of a rare malformation with the cell changes described here gives the syndrome a unique neuropathological profile.

Basal Ganglia↗
PubMed1977

Bladder diverticula and Menkes' syndrome.

Multiple unusual diverticula of the bladder were observed in 3 of 4 children with Menkes' syndrome. This abnormality of the bladder in children with the kiky hair syndrome has only recently been recognized. The diverticula are best visualized on cystographic studies. The clinical manifestation which led to roentgen evaluation of the urinary tract in the 3 children was urinary tract infection or urine retention. Though the etiology of these diverticula is unknown, evidence is presented to suggest that they are acquired.

Brain Diseases, Metabolic↗
PubMed1977

Encephalopathy in children with chronic renal failure.

The progressive encephalopathy observed in 5 children with chronic renal failure was clinically similar to the so-called dialysis encephalopathy of adults, except that it was not related to dialysis therapy. Renal osteodystrophy is more prevalent in children than in adults and often more severe. The attempt to control the crippling deformities of renal osteodystrophy in growing children with renal insufficiency has led to the use of large quantities of aluminum containing antacids. The encephalopathy observed in children with chronic renal failure may be related to the oral ingestion of aluminum containing compounds in the presence of persistent secondary hyperparathyroidism. We suggest that alternative methods for the adequate control of serum phosphorus levels should be sought and indications for parathyroidectomy in children reevaluated. During the past 18 mos we have lowered the dose of aluminum containing compounds to 50 to 100 mg/Kg/day in our patients with progressive renal failure and recommend parathyroidectomy. No new cases of the encephalopathy have occurred.

Age Factors↗
PubMed1976

Cerebellar microgyria.

The histological features of cerebellar microgyria are described in a 15 month old infant. The bizarre appearance of this malformation seems to be the result of two basic factors: secondary fusion between preformed folia, and deficient and disorderly formation of the internal granule cell layer. The findings support the view according to which cerebellar microgyria is probably due to a pathological process involving the external granule cell layer during a relatively late period of cerebellar development.

Autopsy↗
PubMed1976

Neuropathologic changes in ataxia-telangiectasia.

The neuropathologic findings in a 17-year-old boy with ataxia-telangiectasia are described. In agreement with previous reports, pathologic changes were present in the cerebellum, spinal cord, dorsal root ganglia, and straited muscle. The lesions in the spinal cord and dorsal root ganglia were more severe than previously described. Abnormalities were also seen in several brain stem nuclei, including the mesencephalic nucleus of the trigeminus and the substantia nigra. In addition, a small hamartomatous tumor was found in that thalamus.

Adolescent↗
PubMed1975

Copper infusion therapy in trichopoliodystrophy.

The effect of repeated intravenous infusions of cupric salts was studied in two patients (cousins) with trichopoliodystrophy identified at the ages of 3 days and 2 and-one-half mo, respectively. The intravenous doses of copper were gradually increased and normal or near-normal serum values were achieved after 150 mug/Cu2+/kg were administered daily for 5 days. At that time the hepatic concentration of copper in the two patients increased from 14 to 38 mug/gm of dry weight from 7 to 45 mug/gm of dry weight, respectively, and the muscle homogenate exhibited the capacity to oxidize pyruvate-3-14C. Continued infusions of cupric salts in doses of 190-220 mug/kg/day once or twice weekly were necessary to maintain elevated hepatic and serum concentrations of copper. At 6 mo of age the younger infant had reached a functioning level of 4 mo. The older infant demonstrated progressive loss of neurologic functions and died at 15 mo of age. These observations suggest that the neurologic and biochemical dysfunctions of trichopoliodystrophy may be altered by infusion of cupric salts early in the course of the disease.

Age Factors↗