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Biomedical subjects

M Nigro

Publications and source records attributed to M Nigro.

At least 55 records · Page 3Linked to original sources

The renal lesions of tuberosclerosis (cysts and angiomyolipoma)--screening with sonography and computerized tomography.

The two most common sonographic abnormalities in the kidneys of 23 tuberous sclerosis (TS) patients ranging in age from newborn to 30 years are angiomyolipomas (12/23) (AML) and renal cysts (10/23). These usually both occur in the same patient with only 9 cases (39%) having sonographically normal kidneys. Of the 14 affected patients, 2 had cysts without AML and 4 others had AML without cysts. The sonographic appearance of an AML varied from a large 6 cm solid mass with little increased echogenicity (1/12) to subtle small (4 mm) extremely echogenic regions in the periphery of the kidney (11/12). The sonographic appearance of the cysts were anechoic lesions varying in size from 2 mm to 2 cm with thin uniform posterior walls and posterior enhancement. Renal lesions are found more frequently with increasing age. Sonography is the preferred screening procedure for the renal lesions of T.S.

Adolescent↗

Prevalence, ultrastructure of the cyst wall and infectivity for the dog and cat of Sarcocystis sp. from fallow deer (Cervus dama).

The prevalence of Sarcocystis sp. (Protozoa: Sarcocystidae) in fallow deer (Cervus dama) in Tuscany, Italy was determined by digestion technique and histological examination. Forty-four of 45 fallow deer were infected. Infections occurred in adult deer and in fawns. Samples from the heart were more intensively parasitized than samples from tongue, oesophagus and diaphragm muscle. With transmission electron microscopy, the primary cyst wall was folded and formed narrow, overlapping, sinuous projections which were often parallel to the cyst surface. Dogs fed heart samples from infected fallow deer shed sporocysts after 10-11 days. Cats fed the same samples did not shed any sporocysts.

Animals↗

The cerebral electrical fields in cerebellar syndrome.

Cerebral electrical field mapping in a patient with a clinically pure cerebellar syndrome showed the field maximum at the Pz electrode position. Flash visual evoked potentials also showed, in addition to the occipital response, a late positive peak at 344 ms at Pz. The findings suggest that the Pz electrode may at times record far field activity from the cerebellum.

Cerebellar Ataxia↗

Phosphorylation of intact erythrocytes in human muscular dystrophy.

The uptake of exogenous 32Pi into the membrane proteins of intact erythrocytes was measured in 8 patients with Duchenne muscular dystrophy. No abnormalities were noted after autoradiographic analysis. This contrasts with earlier results obtained when isolated membranes were phosphorylated with gamma-[32P]ATP, and suggests a possible reinterpretation of those experiments.

Adolescent↗

Congenital obstructive uropathy and nodular renal blastema.

The occurrence of nodular renal blastema and renal dysplasia was determined in a retrospective study of 75 cases of congenital obstructive uropathy. Nodular renal blastema was present in 3 upper pole nephrectomy specimens removed as a consequence of nonfunction owing to ectopic ureterocele; none was dysplastic. A more differentiated type of nodular renal blastema was present in 3 other total nephrectomy specimens, bilateral involvement in a case of posterior urethral valves and unilateral nodular renal blastema associated with ureteral atresia. This subset of differentiated nodular renal blastema was associated with renal dysplasia.

Child↗

Peroxisomal defects in neonatal-onset and X-linked adrenoleukodystrophies.

Accumulation of very long chain fatty acids in X-linked and neonatal forms of adrenoleukodystrophy (ALD) appears to be a consequence of deficient peroxisomal oxidation of very long chain fatty acids. Peroxisomes were readily identified in liver biopsies taken from a patient having the X-linked disorder. However, in liver biopsies from a patient having neonatal-onset ALD, hepatocellular peroxisomes were greatly reduced in size and number, and sedimentable catalase was markedly diminished. The presence of increased concentrations of serum pipecolic acid and the bile acid intermediate, trihydroxycoprostanic acid, in the neonatal ALD patient are associated with a generalized diminution of peroxisomal activities that was not observed in the patient with X-linked ALD.

Adrenoleukodystrophy↗

Electrophysiologic studies in neonatal adrenoleukodystrophy.

Recent electrophysiologic studies have focussed attention on the X-linked adrenoleukodystrophy (ALD) and its myeloneuropathic variant. No organized studies are, however, yet available on its relatively recently described neonatal variant. We conducted electroencephalographic, electroretinographic and evoked response studies in 2 patients with neonatal ALD. In one patient, an infant, initial EEG, hypsarrhythmic in waking and somewhat periodic in sleep, showed dramatic improvement on ACTH therapy accompanied with a seizure-free status. The EEG and clinical improvement, however, were temporary. No improvement occurred following pyridoxine therapy. Her electroretinogram (ERG), visual evoked responses (VERs) and far-field short latency brain-stem auditory evoked responses were also abnormal. The latter studies probably reflected photoreceptor degeneration, optic nerve involvement, cochlear and/or auditory nerve involvement respectively in neonatal ALD. The other patient, a 3.5-year-old girl, also had an EEG characterized by a moderate- to high-amplitude slow background activity and high-amplitude multifocal, generalized or periodic paroxysmal discharges, but presence of some better formed theta frequencies in background activity precluded a hypsarrhythmic label on her EEG. Her ERGs and VERs were totally extinct but in contrast to the first patient, she had clear-cut optic atrophy and retinitis pigmentosa on ophthalmological examination.

Adrenoleukodystrophy↗

An ultrastructural study of oogenesis in a marine triclad.

The ultrastructural features of oocyte differentiation were studied in the marine triclad Cercyra hastata. Oocytes at several stages of maturation, each surrounded by follicle cell projections, are present within each of the two ovaries. A pre-vitellogenic and a vitellogenic stage have been detected in the oogenesis of C. hastata. The pre-vitellogenic stage is mainly characterized by an increase in the nuclear and nucleolar volume and activity, and the appearance and development of cortical granule precursors which are elaborated by the Golgi complex. In early phases of the vitellogenic stage, intense delamination and blebbing of the nuclear envelope occurs which probably contributes to an increase in number of cytoplasmic membranes and to transfer of nuclear material to the cytoplasm. The rough endoplasmic reticulum is extensively developed an often assumes a 'whorl' array. Several areas of yolk precursor formation appear in the whorls. Numerous 2-5 micrometers protein yolk globules are subsequently formed which appear surrounded by a double membrane (cisternae of the smooth endoplasmic reticulum) and become randomly distributed throughout the cytoplasm of mature oocytes. The peripheral ooplasm is occupied by a monolayer of electron-dense cortical granules. Finally, the evolutionary significance of the autosynthetic mechanism of yolk production is discussed.

Animals↗

Screening for metabolic disease in a metropolitan hospital.

Screening for metabolic diseases at Children's Hospital of Michigan, Detroit during 1978 and 1979 led to the discovery of 7.5 cases per year, representing a marked increase over previous years. Five cases of organic aciduria were identified during this two-year period by use of urinary gas chromatography. Four of these were found to have methylmalonic aciduria. The increase in detection rate was due to the addition of an organic acid screening technique and greater use of two standard screening tests. The yield of screening by these two tests also improved, which we attribute to the better use of specific criteria. Inclusion of a simple urine screening test for methylmalonic acid is recommended in the workup of infants with episodic vomiting, lethargy, acidosis, or catastrophic illness.

Amino Acid Metabolism, Inborn Errors↗

Transient intellectual and psychosocial regression during recovery phase of stage V Reye's syndrome.

The outcome of stage V Reye's syndrome survivors has not been critically assessed. Three teenage survivors, who initially exhibited severe psychosocial and intellectual regression, are described. Findings support the observation that the neurological outcome from Reye's syndrome correlates with the duration of altered consciousness during the acute phase. Recovery of intellectual, psychosocial, learning and motor function is reported. A period of transient dementia was noted, which may be typical in recovery from stage V Reye's syndrome. Unlike other types of encephalopathies, complete resolution may be expected.

Adolescent↗