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Biomedical subjects

Z Neeman

Publications and source records attributed to Z Neeman.

10 recordsLinked to original sources
PubMed2002

Early diagnosis of primary ciliary dyskinesia in a newborn without situs inversus.

UNLABELLED: Primary ciliary dyskinesia has been reported as a rare cause of respiratory distress during the neonatal period. This diagnosis is readily suspected in cases presenting with accompanying situs inversus. The aim of this study was to report on a pair of siblings with primary ciliary dyskinesia. The first case was an infant diagnosed with primary ciliary dyskinesia at the age of 14 d despite lack of situs inversus. The infant had presented with respiratory distress and atelectasis almost immediately after birth. The sibling, born one year later, presented with situs inversus, therefore allowing diagnosis of primary ciliary dyskinesia to be made immediately after birth. CONCLUSIONS: Diagnosis of primary ciliary dyskinesia should be considered in newborns presenting with respiratory distress or atelectasis. Early institution of an adequate treatment programme and follow-up may reduce or prevent further complications of the disease.

Biopsy, Needle↗
PubMed1997

Fracture of the occipital condyles and associated craniocervical ligament injury: incidence, CT imaging and implications.

Thin section contiguous axial computer tomography (CT) was used to demonstrate fractures of the occipital condyle and craniocervical ligament injury. During a 12-month period, 55 consecutive patients who had sustained high energy blunt trauma to the head or upper cervical region were examined (38 male and 17 female, age range 3-80 years, median 24 years). If occipital condyle fracture was suspected clinically, CT was performed from C2 to the foramen magnum with two dimensional sagittal, coronal, and curvilinear reconstructions and employing bone and soft tissue windows. Occipital condyle fractures were classified according to Anderson and Montesano types 1, 2 or 3. Injury to the internal craniocervical ligaments was described. Nine of 55 patients had occipital condyle fractures (16.4%). Injury of the alar ligaments was demonstrated in four and tectorial membrane injury in two patients. Three of the nine patients had associated fractures of cervical vertebrae. Five of nine patients had a normal Glasgow coma scale on admission (55%) and in two patients the occupital condyle fracture was the only significant injury. Plain cervical radiographs were non-diagnostic. Two patients had significant pain and limited motion of the craniocervicum several months following injury. In conclusion, CT should be performed where there is a high clinical suspicion of occipital condyle fracture, that is based mainly on the mechanism of injury.

Adolescent↗
PubMed1996

Occipital condyle fracture and ligament injury: imaging by CT.

The true incidence of fracture of the occipital condyles is unknown. It may be associated with instability at the craniocervical joint. CT is the modality of choice for the demonstration of these fractures, but its use for imaging of the associated ligament injury has not been reported. In order to demonstrate normal anatomy, occipital condyle fracture and ligament injury, and to estimate the incidence of this lesion, 21 children and young adults with high-energy blunt craniocervical injury were examined prospectively. Thin-slice, axial, contiguous, CT was performed from the base of C2 to above the foramen magnum. Bone and soft tissue windows and coronal, sagittal, and curvilinear 2D reconstructions were performed. Five occipital condyle fractures were identified in four patients (19 %), with demonstration of alar ligament injury in two cases and local hematoma in one. In four, artifacts or rotation precluded assessment of ligaments. In all remaining cases normal bone and ligament anatomy was demonstrated. Fracture of the occipital condyles following craniocervical injury is not uncommon in children and young adults. Normal bone and ligament anatomy and pathology can be safely and clearly demonstrated in seriously injured patients and others using this CT technique. Increased awareness of this entity and a low threshold for performing CT should avoid the potentially serious consequences of a missed diagnosis.

Adolescent↗
PubMed1993

The relationship between plasma and dialysate lipoproteins and apoproteins in patients treated by continuous ambulatory peritoneal dialysis.

End-stage renal disease is frequently associated with lipoprotein abnormalities, manifested primarily by elevated very low-density lipoprotein levels combined with a decrease in high-density lipoprotein levels. These lipoprotein disturbances are further exacerbated in continuous ambulatory peritoneal dialysis. We examined the lipoprotein and apolipoprotein profiles in the blood and dialysate effluents of eight normolipidemic and five hypertriglyceridemic patients with end-stage renal failure treated with continuous ambulatory peritoneal dialysis. The normolipidemic patients were found to have significantly greater losses, as expressed by the fractional catabolic rates through the dialysate, for protein, total cholesterol, and very low-density lipoprotein cholesterol. These results suggest that the hypertriglyceridemia associated with continuous ambulatory peritoneal dialysis may be mitigated in some patients by the excessive loss of very low-density lipoprotein, or some other plasma constituent, into the dialysate effluent.

Apolipoproteins A↗
PubMed1992

[Infantile neuroaxonal dystrophy].

Infantile neuroaxonal dystrophy is a degenerative disease, mostly of nerve endings in the central and peripheral nervous system, with typical pathological findings. We describe a 3-year-old boy who had suffered from nystagmus, progressive muscle weakness and dementia since the age of 10 months. Biochemical work-up, MRI of the brain, and electrophysiological studies were within normal limits. Only skin biopsy, and sural nerve biopsy at a later stage, were diagnostic. We stress the importance of performing tissue biopsy even when intensive investigation is negative.

Biopsy↗
PubMed1991

[Prenatal diagnosis of albinism].

Prenatal diagnosis of oculocutaneous albinism (OCA) was made in 1 of 6 pregnancies at risk examined during the 20th week of gestation. A skin biopsy was taken from the fetal scalp under ultrasonic screening. Light and electron microscopy studies were performed in each case to demonstrate melanin pigment and melanosomal development in the melanocytes of the hair bulbs and the epidermis. In 1 fetus albinism was diagnosed by the absence of melanin pigment and by the demonstration that melanosomes were only present in stages I and II. In the other 5 fetuses melanin pigment and mature melanosomes (up to stage IV) were demonstrated. The pregnancy with the albino fetus was interrupted and the diagnosis of OCA was confirmed at autopsy.

Albinism, Oculocutaneous↗
PubMed1988

Comparative morphology and tumourigenicity of human hepatocellular carcinoma cell lines in athymic rats and mice.

Four human hepatoma cell lines PLC/PRF/5, Hep G2, Sk-Hep 1 and Mahlavu were inoculated subcutaneously into athymic Balb/c nude mice and N/NIH outbred nude rats, producing well encapsulated tumours. The 4 hepatoma tumour types in the athymic rodents differ morphologically. PLC/PRF/5 and Hep G2 cells are well differentiated polygonal cells which resemble normal hepatocytes. Tumour arrangement is characterized by solid masses and trabeculae while stromal support is minimal. In contrast, Mahlavu and Sk-Hep 1 tumours have a sarcomatous appearance and consist of spindle shaped cells arranged in solid masses with a rich stromal support. Tumourigenicity of hepatoma cells in the athymic rodents was dependent on injected cell type, inoculation density, relative immunocompetence of the host and the species of animals used. In nude mice, Sk-Hep 1 cells were the most tumourigenic, while Hep G2 cells were tumourigenic only at very high inoculation densities. In nude rats, which were more resistant to tumour formation, PLC/PRF/5 cells were the most tumourigenic. Pre-treatment of athymic mice and rats with total body irradiation resulted in enhanced tumourigenicity for all hepatoma cell lines tested. This was manifested as increased "take" rates, a decreased latency from tumour cell injection to tumour detection, increased tumour weight, and for PLC/PRF/5 cells an increased invasiveness to adjacent body cavities. Furthermore, following irradiation, the minimal number of injected cells required to produce subcutaneous tumours was markedly reduced in both animal species, regardless of tumour cell type. The protocols described enable the reproducible growth of human hepatoma tumours in athymic rodents.

Animals↗
PubMed1983

Successful treatment of autoimmune manifestations in MRL/l and MRL/n mice using total lymphoid irradiation (TLI).

The autoimmune manifestations of MRL-+/+ (MRL/n) and MRL/Mp-lpr/lpr (MRL/l) murine models of systemic lupus erythematosus (SLE) were successfully reversed following total lymphoid irradiation (TLI) therapy consisting of 8-12 daily fractions of 200 rad. Following radiotherapy the characteristic lymphadenopathy of MRL/l disappeared, proteinuria was 334 mg% compared to a peak of 2272 mg% in untreated controls, and the median survival time was prolonged to 423 days compared to 214 days in untreated mice. The albuminuria of TLI-treated MRL/n mice was 194 mg% compared to 1180 mg% in untreated controls. The survival of treated MRL/n mice was prolonged to a median of 389 as compared to 190 days in untreated controls. The effect of TLI on antiDNA antibodies in both MRL/l and MRL/n was less remarkable. However, the antiDNA activity reached normal levels in most long-living mice. The most impressive finding was complete reversal and/or prevention of the SLE-like glomerulonephritis in MRL/l mice as documented by light and electron microscopy. Immunomanipulation with TLI should be further evaluated as a possible treatment modality in intractable human autoimmune disorders.

Animals↗
PubMed1983

Scanning immuno-electron microscopy of a monoclonal, Epstein-Barr Virus(EBV) transformed human cell line producing rheumatoid factor in-vitro.

Selected lymphocytes from a patient with rheumatoid arthritis were infected with Epstein-Barr virus (EBV) and the emerging cell line (RF-AN) has now been successfully maintained in culture for more than two years. Multiparameter studies, including surface markers, ultra-structure and scanning immuno-electron microscopy, were utilized to characterize and evaluate this unique human cell line. Like other B-lymphoblastoid cell lines, RF-AN cells display multiple microvilli, contain surface and intracytoplasmic immunoglobulin (IgM lambda) and continuously produce monoclonal IgM lambda in vitro. The latter serves as an autoimmune antibody directed against IgG and is termed rheumatoid factor (RF). When RF-AN cells were incubated with human or rabbit IgG conjugated to polystyrene (latex) microspheres, almost all cells were labeled with the marker. However, cells did not label with goat IgG conjugated to latex. RF-AN cells were also labeled with latex microspheres coupled to goat antisera directed against human IgM or lambda chains but not with goat anti-human IgG or anti-human kappa chains. It is suggested that this mode of immunomicroscopy is reliable and may provide more useful data than other standard immunological techniques.

Antibodies, Monoclonal↗