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K Maurseth

Publications and source records attributed to K Maurseth.

15 recordsLinked to original sources
PubMed2001

Neonatal lethal dwarfism with distinct skeletal malformations--a separate entity?

We describe a case of neonatal lethal dwarfism characterised by short trunk, short, stick-like tubular bones, deficient ossification of the axial skeleton and broad, sclerotic horizontal ribs. Two similar cases have previously been reported as examples of the Neu-Laxova syndrome. However, the radiological findings of the Neu-Laxova syndrome, as reported in 16 out of 40 documented cases, show a heterogeneous pattern of minor features, which differ distinctively from those found in the previous two cases and by us. A literature research did not reveal similar cases, and we therefore suggest that our case, together with the two previous cases, may represent a new distinctive form of neonatal lethal dwarfism.

Bone Diseases, Developmental↗
PubMed1986

Duodenal duplication--a case with special features.

A duodenal duplication almost as large as the stomach proper was found along the greater curvature of the stomach in a 6-month-old Caucasian girl. There was a wide communication with the proximal part of the duodenum, from which the duplication blood supply originated. The duplication and the stomach shared the serosal lining, but had individual, full thickness walls. The duplication was removed in toto, and the communication with the duodenum was closed by transverse suture. The luminal lining of the duplication consisted of corpus type of gastric mucosa. There was a 1 X 1 cm mucosal ulceration in the region of which respiratory tract elements were found. The condition is interpreted as a duodenal duplication with ectopic gastric mucosa and respiratory tract elements. To our knowledge, the occurrence of respiratory tract elements in an intestinal duplication BELOW the diaphragm is being reported for the first time. It offers a challenging explanation for the ulceration found within this duplication.

Barium Sulfate↗
PubMed1982

Vitamin D metabolism in hypophosphatasia.

A 4-month-old boy with the infantile form of hypophosphatasia was followed for 9 months with measurements of serum calcium, phosphate, alkaline phosphatase and various vitamin D metabolites, together with urinary excretion of cyclic AMP. During the initial hypercalcemic stage the serum concentration of 25-hydroxyvitamin D was normal. Urinary cyclic AMP was low and the serum concentration of the dihydroxymetabolites of vitamin D were appropriate to the high serum calcium with low 1,25-(OH)2D and relatively high 24,25(OH)2D and 25,26(OH)2D levels. Due to restrictions of the vitamin D intake and lack of exposure to sun he developed vitamin D-deficiency rickets at 9 months of age with very low serum concentration of 25-hydroxyvitamin D and markedly increased urinary excretion of cyclic AMP. Following vitamin D treatment the serum level of 1,25(OH)2D showed a brisk rise to a considerably elevated value. Initially the serum concentration of alkaline phosphatase was well below the normal range, rose markedly during the stage of active rickets and returned to the characteristic low levels of hypophosphatasia with healing of the rickets.

Calcium↗
PubMed1979

A familial syndrome of diaphyseal cortical thickening of the long bones, bowed legs, tendency to fracture and icthyosis.

The clinical and radiological features of a dominant bone disorder found in six members of the same family are described. The bone disorder was mainly characterized by endosteal cortical thickening of long tubular bones and bowing of the weight-bearing ones. The clinical symptoms were waddling gait, muscle weakness and leg pains, and there was a tendency to fractures. All affected individuals had icthyosis of slight or moderate degree.

Adult↗
PubMed1978

Tracheal compression by the aortic arch following right pneumonectomy in infancy.

Uneventful pneumonectomy was carrried out in a 10-month-old infant for hypoplastic right lung associated with esophageal origin of the right main bronchus. Eight months after operation, symptoms of tracheal compression began. This compression was due to the aortic arch, which was stretched across the lower trachea following the displacement of the heart to the posterior right chest after pneumonectomy. Successful relief of the airway obstruction was achieved by inserting a 20 mm woven Dacron graft between the ascending and the descending aorta, with division of the aortic arch between the left carotid and the left subclavian arteries. An aortogram performed two years after operation confirmed good patency of the graft and normal flow through the descending aorta. The child remains well two years after operation but continues to have mild residual tracheomalacia and limited exercise tolerance, compatible with the presence of only one lung.

Airway Obstruction↗