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Biomedical subjects

K Stehr

Publications and source records attributed to K Stehr.

At least 55 records · Page 3Linked to original sources

[Chronic hemorrhagic pancreatitis in gallbladder polyposis as an initial symptom of metachromatic leukodystrophy].

A 4 1/2 year old boy without previous neurologic disorders developed chronic hemorrhagic pancreatitis and was shown to have polyposis of the gallbladder. Neurologic symptoms emerged at the age of 5 years. The sonographic pattern of an echogenic gallbladder was suspect of metachromatic leukodystrophy. The definitive diagnosis was made by the findings of very low arylsulfatase A activity in the white blood cells and deposits of sulfatides in the stroma of the polyps of the gallbladder.

Cerebroside-Sulfatase↗

[An unusual case of invagination].

Intussusception is an usual pediatric problem in the first two years of life. Nevertheless, it may occur in older children as well, but then often with an uncharacteristic history and atypical x-ray findings. Even symptoms lasting over weeks or months do not exclude intussusception. Ultrasonography is a useful diagnostic approach. In difficult cases endoscopy is indicated.

Child↗

Extreme variant of septo-optic dysplasia.

A newborn female is demonstrated, the first child of healthy unrelated parents who was born after an uneventful pregnancy. Computerized tomography of the brain revealed gross malformations of the parietal and occipital lobes, of the cerebellum and of midline structures. The pictures are reminiscent of hydranencephaly. The association with bilateral coloboma of the papilla and diabetes insipidus is considered an extreme variant of septo-optico dysplasia.

Brain↗

The effect of L-carnitine-supplemented total parenteral nutrition on tissue amino acid concentrations in piglets.

Miniature piglets underwent total parenteral nutrition (TPN) with and without L-carnitine supplementation during a 7-day period. Thereafter the tissue amino acid concentrations of liver, heart, skeletal muscle and brain were determined and compared to those of orally fed animals. The altered tissue amino acid concentrations during TPN without carnitine returned to normal when L-carnitine was supplemented. The most striking changes of tissue concentrations showed taurine in liver, muscle and brain and ethanolamine in heart and brain. In muscle the branched-chain amino acids were increased when L-carnitine was added to the TPN regime. Ethanolamine changes were discussed with respect to the position of this amino acid in the synthesis of phospholipids. The marked decrease of brain taurine concentrations after carnitine-free TPN was accompanied by reduced values for GABA. Both the substances function as inhibitory transmitters in the brain and should be considered when seizure activity in patients with systemic carnitine deficiency is discussed.

Administration, Oral↗

[Sonographic detection of subdural effusions in infancy].

Using the open fontanelle as an acustic window, small frontoparietal subdural effusions are often missed on conventional sagittal or coronar ultrasonographic sections. Using horizontal and frontal skull sections during the first 12 months of life intracranial structures can equally be visualized. In a study of 14 infants aged 6 days to 11 months gray scale ultrasonography was compared to computed axial tomography. The results suggest that horizontal and frontal ultrasonographic skull sections are equally efficient to localize even small subdural effusions without the adverse risks of radiation.

Brain↗

[Diagnosis and clinical course of cerebral hemorrhage in infants based on sonography].

Infants of very low birth weights (less than 1500 g) born before the 32nd week of gestation have a high incidence of neonatal intracranial hemorrhage (ICH). Beside perinatal and postnatal asphyxia the main risk factor for ICH is the immaturity of the infant. Mild intracranial hemorrhages resolve within a few weeks. Severe ICH, which were seen in very immature babies before 30 weeks of gestation and with birth-weights below 1250 g had a fatal outcome. The mortality rate in severe ICH was 50%. The infants which survived severe ICH developed posthemorrhagic hydrocephalus (PHH). Using the open fontanelle as an acoustic window gray scale ultrasonography of children's brain is able to visualize ICH and PHH. We suggest early sonografic brain investigation within the first days of life in all prematurely born infants with perinatal asphyxia. Infants with severe ICH should have weekly sonografic controls, to detect PHH as soon as possible.

Age Factors↗

[Cervical lymphoma from the pediatric viewpoint].

On the background of the immunological maturation of the child, the enlargement of the lymphatic organs are discussed according to their occurrence . Space occupying lesions of the anterior and posterior neck triangles are differentiated facing the favoured occurrence of malignous diseases in the posterior neck triangle. Malignous diseases are found in 15 percent of patient with lymph node swelling. The following tumors of the neck are found in children: Thyreoglossus cysts 26,2%, branchiogenic cysts 23,2%, lymphangioma 10,2%, nonspecific lymphadenopathy 9,8%, tuberculous lymphadenitis including cases caused by mycobacterium avium strains 6,7%, neural tumours 4,1%, thyroid tumours 4,1%, lymphoma and Hodgkins disease 7%, other nonmalignous tumours 6,7%, tumours of the parotid gland 1,5%. Important diseases in differential diagnosis of generalized lymphadenopathy are discussed, as well as prognosis and therapeutical implications.

Age Factors↗

[Clinical and chemical factors in kidney calculus formation in childhood. An analysis of 40 patients].

During 5 years, between 1978 and 1982, 40 patients (22 male, 18 female) aged 1 to 17 years presented with kidney stones at the Erlangen University Children's Hospital. Stone analysis showed calcium oxalate in 61%, calcium phosphate respectively magnesiumammonium phosphate in 33% and cystine in 6%. Calcium phosphate/ magnesiumammonium phosphate stone bearers were most frequently found in the first 5 years of age and showed more often urinary tract malformations, staghorn renal calculi, urinary tract infections and a higher urinary pH. Calcium oxalate stone bearers were more frequently found in patients of school age and had more often relatives with nephrolithiasis. In the group of calcium oxalate stones hypercalciuria, hyperoxaluria and hyperuricosuria were most frequently observed.

Adolescent↗

[Animal experiment studies on the changes in lipid and protein metabolism in L-carnitine-supplemented total parenteral nutrition].

The influence of i.v. L-carnitine on parameters of lipid- and nitrogen metabolism was studied during total parenteral nutrition of mini pigs (x: 4077; n = 9). The infusion protocol was divided into isocaloric and isonitrogenous 48-hour-periods. Amino acids (3 g/kg/day) were administered throughout all three periods. 140 Cal/kg/day were given as non-protein calories, consisting only of glucose during period 1. During periods 2 and 3 an amount of glucose calorically equivalent to 4 g fat/kg/day was substituted with a lipid emulsion. In period 3, L-carnitine (1,5 mg/kg/day) was added. During the entire regime key parameters of fat and nitrogen metabolism were determined. During all three periods indirect calorimetry was performed and the respiratory quotient calculated. The results demonstrate a more effective lipolysis and oxydation of fatty acids during L-carnitine supplementation. This results in an increased energy gain from exogenously administered fat and a distinct improvement of nitrogen balance.

Amino Acids↗

[A prolactin-producing microadenoma as the cause of galactorrhea and delayed puberty in an adolescent boy].

The case history of a 16 years old boy with delayed puberty and the sudden onset of galactorrhea is presented. Prolactin analyses and X-ray studies of the sella turcica lead to the diagnosis of a pituitary tumor. A prolactin producing microadenoma was selectively excised via the transsphenoidal route. Serum prolactin resumed normal concentrations within hours after the operation. During the next months the boy showed the signs of normal puberty with increasing concentrations of serum testosterone. Because serum prolactin concentrations remained within the normal range, therapy with bromocriptine was not necessary. A spermiogram however, performed seven months after the operation, showed signs of a viscosipathy and terato-asthenozoospermia.

Adenoma↗

[Early diagnosis of congenital metabolic diseases].

Diseases of metabolism may lead to irreversible organ damage before a typical pattern of clinical symptoms leads to diagnosis. Because of the possibility of treatment there is an urgent need for an early diagnosis to prevent any damage. Successful screening procedures, like the "Guthrie test" for phenylketonuria and TSH-screening for hypothyroidism are presented. Screening for Duchenne's progressive muscle dystrophy and for cystic fibrosis is discussed and evaluated. Attention is drawn to a screening for familial hypercholesterolemia, which might be important in the future.

Cystic Fibrosis↗