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Biomedical subjects

K Katz

Publications and source records attributed to K Katz.

At least 19 recordsLinked to original sources
PubMed1992

Long-term follow-up of partial splenectomy in Gaucher's disease.

Seven children with Gaucher's disease who underwent partial splenectomy were followed for 7 to 8 years. None of the children had systemic sepsis or symptoms related to liver enlargement. Bone crisis occurred in only two children who had experienced bone crisis prior to partial splenectomy. This contrasts with the development of bone crises in five of six children who underwent total splenectomy at a similar age and who had previously been free of bone symptoms. Partial splenectomy should be regarded as a temporary solution in the treatment of hypersplenism and the mechanical compression related to the huge spleen. In 71% of the patients, massive enlargement and a severe to moderate degree of pancytopenia occurred again after 3 to 8 years; total splenectomy was required in three patients.

Adolescent↗
PubMed1992

Non-specific X linked mental retardation with aphasia exhibiting genetic linkage to chromosomal region Xp11.

A new type of non-specific X linked mental retardation is described in a three generation family. The three affected males had severe mental retardation (IQ 20 to 30), mutism, growth failure, frequent infections, seizures, and the following minor anomalies: brachycephaly, frontal hair whorl, square face, large mouth, thick lips, and prognathism. There was not a characteristic facies. Normal laboratory studies on the proband included a karyotype with fragile X screening, skeletal survey, blood amino acid, urine organic acid, and HGPRT levels. Linkage analysis was performed with 10 X chromosome DNA probes of which probe DXS255 at chromosomal region Xp11.22 gave a maximal two point lod score of 2.10 if phase was inferred and 1.20 if it was not. Crossovers were shown with probes mapping to regions Xp22, Xp21, and Xq28. Comparison of these patients with 80 X linked causes of mental retardation, including 41 which might be classified as 'non-specific', showed no other disorders compatible with the phenotypic and linkage data.

Abnormalities, Multiple↗
PubMed1991

Hemorrhage associated with "bone crisis" in Gaucher's disease identified by magnetic resonance imaging.

Children suffering from Gaucher's disease were examined by magnetic resonance imaging (MRI) during a characteristic episode of "bone crisis". An unexpectedly high intramedullary as well as subperiosteal signal was observed on both the T1- and T2-weighted sequences in 5 patients, suggesting a subacute hemorrhage or hematoma. It is conceivable that such a painful hemorrhage is an important component of the "bone crisis" phenomenon. Furthermore, in these cases this is a specific sign which may enable differentiation of bone crises from other types of bone pain associated with Gaucher's disease.

Adolescent↗
PubMed1991

Normal ranges of hip motion of preterm infants.

The hip motion of 158 preterm infants with gestational ages between 25 and 36 weeks was measured. The results of this study, compared with those of previous studies of term infants, show that hip motion changes with increasing gestational age above 25 to 27 weeks.

Female↗
PubMed1991

Bone scans in the diagnosis of bone crisis in patients who have Gaucher disease.

Of thirty-four occurrences of bone pain in seventeen children and young adults who had type-I Gaucher disease, twenty-five were finally diagnosed as bone crises. On the basis of a bone scan with technetium-99m methylene diphosphonate, a correct diagnosis of bone crisis was made for twenty-three occurrences, with a sensitivity of 0.92. At the onset of a crisis, the bone scan typically showed decreased uptake of radionuclide at the involved site. After six weeks, a repeat bone scan of the long bones showed a ring of increased uptake surrounding an area of decreased uptake. At six months, the appearance of the bones on the radionuclide scan was normal.

Adolescent↗
PubMed1990

PEG 400, a hydrophilic molecular probe for measuring intestinal permeability.

There is a widely held misconception that low-molecular-weight polyethylene glycols are "highly lipophilic" permeability probes and therefore are transported across lipid cell membranes. The relative lipophilicity of polyethylene glycols 400 and 600 were examined by determining their partition coefficients (Kd) in water and organic solvents of increasing relative polarity. The Kd of polyethylene glycol 414 between hexane and water was 0.000015, indicating that there are only 1.5 parts of polyethylene glycol 414 in hexane for 100,000 parts of polyethylene glycol 414 in water. When the Kd was determined in organic solvents with increasing relative polarity or "water character", there was a linear increase in Kd. The relative urinary recovery of individual molecular weight fractions of polyethylene glycol 400 in normal volunteers was analyzed. After oral ingestion, there was a progressive decrease in relative urinary recovery of increasing molecular weight fractions of polyethylene glycol 400 suggesting that increase in the molecular size limited polyethylene glycol intestinal permeability. There was excellent correlation between the relative urinary recovery and the hydrophilicity of the intravenously administered polyethylene glycol 400 fractions. It is concluded that polyethylene glycols 400 and 600 are strongly hydrophilic. Since partitioning of polyethylene glycol into lipid phase is negligible in lipid/water mixtures, they are unlikely to be transported via lipid pathways. The intestinal permeability of polyethylene glycols are governed by their molecular size, and once in circulation their urinary excretion appears to be governed in part by their plasma or water solubility.

Adult↗
PubMed1989

Humeral lengthening for septic neonatal growth arrest.

A 9-year-old girl was treated by humeral lengthening for a 8.5 cm deficit caused by a septic arthritis 2 days after birth. A Wagner distraction apparatus was used after an osteotomy was done distal to the deltoid insertion. Six centimeters of length were gained. A second lengthening of about 7.5 cm is planned at the end of the patient's growth. No complications were encountered; by dividing the lengthening into two procedures, none are anticipated. At 1-year follow-up, there was a full range of shoulder, elbow, and hand motion; the humeri were virtually the same length.

Arthritis, Infectious↗
PubMed1988

Injuries in attempted suicide by jumping from a height.

This study was undertaken to investigate the pattern of injuries and the management of 28 patients who attempted suicide by jumping from a height. Most of the patients had serious psychiatric disorders. All but two of the patients sustained multiple injuries. The most common injury patterns were fractures of the spine and lower extremities. It was concluded that, in addition to urgent orthopaedic and surgical care, early psychiatric assessment and therapy with transfer to a psychiatric department as soon as possible facilitates the recovery and rehabilitation of these patients.

Adolescent↗
PubMed1988

Seat insert for cerebral-palsied children with total body involvement.

The sitting problems of cerebral-palsied children with total body involvement are discussed. A moulded seat-insert has been devised which resolves most of these problems. Custom-made for each child, the insert incorporates a head-support and the seat components are connected by hinges to allow some range of movement.

Cerebral Palsy↗