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Biomedical subjects

H B Christesen

Publications and source records attributed to H B Christesen.

10 recordsLinked to original sources

[Congenital hyperinsulinism].

In the last five years, our knowledge about the heterogenous syndrome of congenital hyperinsulinism (HI) has expanded explosively. HI may be familiar or sporadic, mild or severe, transitory or persistent, and histologically focal or diffuse. At least 63 disease-causing mutations have been found in the genes for the beta cell's ATP-dependent potassium channel, whose elements are the sulphonylurea receptor, SUR1, and Kir6.2. Other mutations cause enhancement of the glucose-stimulated ATP production in the beta cell. The resulting non-functional, or closed, potassium channel causes hypersecretion of insulin. Genetic screening has succeeded in detecting mutations in less than 50% of HI-patients. Genotype-phenotype relations, diagnosis and treatment are reviewed.

Genetic Testing↗

Pancreatic beta-cell stimulation tests in transient and persistent congenital hyperinsulinism.

UNLABELLED: In congenital hyperinsulinism (HI). the in vivo pancreatic beta-cell function is poorly described. Among 14 neonates with severe hyperinsulinaemic hypoglycaemia, 2 patients had very prolonged or persistent hypoglycaemia and mutation in the sulphonylurea receptor SURI gene. Patient 1 had transient HI and was treated medically for 3.5 mo before clinical remission was seen. He had initially very high basal and stimulated C-peptide and insulin levels, followed by a state of normal preprandial values, but blunted beta-cell glucose sensitivity, before complete beta-cell normalization occurred. A single. paternal SURI mutation, G1382S, was found suggesting focal type HI. Patient 2 had persistent HI and underwent 3 pancreas resections up to the age of 2 y, 7 mo, followed by a state of mild diabetes. On biopsy, diffuse-type beta-cell hypertrophy was seen. The beta-cell response to glucose and glucagon stimulation was blunted before, as well as after, pancreas resections. Compound heterozygosity for the SUR1 mutations 3992-3c to g and N188S was found. CONCLUSION: Transient, possibly focal, HI with paternal SUR1 mutation was associated with a gradual, but complete normalization of the in vivo beta-cell function; in the diffuse type HI, a blunted beta-cell response to glucose and glucagon stimulation persisted. In vivo beta-cell stimulation tests may contribute to the characterization of the HI subtypes.

Child, Preschool↗

Prolonged elimination of tolbutamide in a premature newborn with hyperinsulinaemic hypoglycaemia.

So far, gestational diabetes treated with tolbutamide has never been associated with severe hypoglycaemia in the newborn when the mother's diabetes was well controlled. We report a case of a premature neonate, gestational age 34 weeks, with severe and long-standing hypoglycaemia from birth. The mother had well-controlled gestational diabetes, treated with tolbutamide from the 24th week of gestation until delivery. The neonate had inappropriately high levels of serum proinsulin, insulin and C-peptide relative to blood glucose concentrations. From day 19 after birth, the levels were normalized. Serum tolbutamide was 140.6 micromol/l (38 microg/ml) at 3 h after birth. Zero-order kinetics were seen during the first 90 postnatal hours. The half-life of serum tolbutamide decreased from 46 to 6 h. It is suggested that tolbutamide, when given to the mother until delivery, may cause severe and prolonged hyperinsulinaemic hypoglycaemia in premature neonates. The initially prolonged tolbutamide half-lives and zero-order kinetics suggest immaturity of hepatic elimination during the first 2 days of postnatal life.

Adult↗

Prediction of complications following caustic ingestion in adults.

The records of 86 adults admitted to hospital following caustic ingestion were reviewed. Eighteen patients (21%) developed complications; of these, six were fatal. Patients without symptoms or signs did not develop complications. Complications occurred only following lye ingestion, or intentional ingestion of hydrochloric acid or ammonia water. Of patients with such ingestion, and two signs or symptoms or more, 70% developed complications. Oesophagoscopy did not significantly improve the prediction of complications. There was a strong trend, however, for more severe complications with more severe submucosal, circumferential oesophageal injuries (P = 0.003). The study suggests that only adults with symptoms or signs following strong alkali or strong acid ingestion are at risk of complications. In adults who are symptomatic following ingestion of strong acid or alkali, oesophagoscopy is important to identify the potentially fatal transmural injuries.

Administration, Oral↗

Prediction of complications following unintentional caustic ingestion in children. Is endoscopy always necessary?

The records of 115 children hospitalized following caustic ingestion over an 18.5-year period from 1976 to 1994 were reviewed. The relationship between types of product ingested, signs and symptoms, degree of esophageal injury and complications was analyzed. All complications were the result of strong alkali ingestion (sensitivity = 1.0). Among the 102 incident patients, 36.8% of lye ingestions resulted in complications, whereas only 2.7% (one) of automatic dishwasher detergent (ADD) ingestions caused any complications (p < 0.01). Endoscopy 6 h to 4 days after injury was accurate in predicting or identifying complications in all types of strong alkali ingestions. In lye ingestions, endoscopy was not superior to the test, "one or more signs or symptoms" in predicting complications (predictivity = 1.0). Endoscopy is recommended to establish or confirm a prognosis, or to identify acute respiratory complications, in symptomatic ingestions of lye or ammonia water, in children with respiratory symptoms, and in rare cases of severe symptoms following ADD or strong acid ingestion. It is suggested that children who are non-symptomatic following unintentional ingestions are not at risk of complications and do not need endoscopic examination.

Adolescent↗

[Diagnosis and treatment of caustic ingestion].

The acute diagnostic procedures in caustic ingestion are primarily directed towards the recognition of life-threatening complications such as airway obstruction, perforation of inner organs and, in severe acid ingestion, metabolic acidosis. The endoscopic injury grading forms the basis of a differentiated treatment. Not all patients need endoscopic evaluation; the indications are discussed. The treatment is controversial. Fluid given orally is recommended within few minutes of ingestion, and thus rarely administered at the arrival to the hospital. Here, the task is to secure the vital functions and to relieve pain. The prevention of oesophageal stricture in deep, (near)-circumferential ulcerations is essential for the further treatment. Once-formed strictures are usually treated with dilatation.

Burns, Chemical↗

Epidemiology and prevention of caustic ingestion in children.

A total of 102 children less than 16 years of age admitted for caustic ingestion in the period 1976-1991 were registered. The annual incidence rate of hospitalization was 10.8:100,000 for the city of Aarhus, Denmark. Esophageal burns occurred with a frequency of 5.0:100,000 per year. Ninety-four percent of the children were less than 5 years old. For this age group, the incidence rates of admission and esophageal burns were 34.6:100,000 and 15.8:100,000, respectively. All ingestions were accidental. The incidence rates of esophageal burns in children 0-4 years old (p = 0.019) decreased significantly during the period studied. The cause of this decrease is not clear, but a change in the spectrum of household products and the gradual introduction of child-proof caps are possible explanations. To minimize the frequency of accidents, an information campaign directed specifically at parents of toddlers is recommended. Information material should stress that caustics should always be inaccessible to children and stored separately, and should never be decanted.

Accident Prevention↗

Caustic ingestion in adults--epidemiology and prevention.

A 16 year retrospective review identified 179 patients hospitalized for ingestion of caustic products in Aarhus County, Denmark. Seventy-five were adults over 15 years of age and residents of Aarhus County. The average annual incidence rate of hospitalization for caustic injury in adults was 1/100,000 with an incidence of esophageal burns of 0.8/100,000/y. Of the adults 61% attempted suicide: of these 61% were women. The annual incidence rate for adult women attempting suicide had an increasing trend (p = 0.048). Changes of the incidence rate for unintentional ingestions and for male suicide attempts were not significant. More than half of the suicide attempts were by patients with a history of a psychiatric illness. Of the caustics ingested by adults 94% were liquids. Acids accounted for 55% of the ingested products. Suicidal ingestion of hydrochloric acid was fatal in 6 of 12 adults. The inavailability of liquid caustics in the homes of patients at suicidal risk might prevent impulsive ingestion.

Adolescent↗

[Ingestion of caustic agents. Epidemiology, pathogenesis, course, complications and prognosis].

The incidence of caustic ingestion varies with the availability of caustic products and preventive measures. A transient increase was seen in the US round 1970, in Denmark in the late seventies. Diagnosis and treatment of caustic ingestion injuries remains controversial, and must to a high degree be based on retrospective studies and animal studies, especially concerning the pathology and clinical course of the disease. These subjects are reviewed together with complications and prognosis. It is stressed, that ingestion of alkalies and acids result in a variety of lesions, depending on a number of factors listed. Ingestion of alkalies occurs in approximately 85% of all cases, with oesophageal stricture as the most frequent complication, and tracheal necrosis as the most frequent cause of death. Ingestion of acid in large amounts may lead to early gastric perforation, massive metabolic acidosis, and eventual death. Pyloric stenosis is the most common complication of acid ingestion. Approximately 33% of all patients admitted with (suspected) caustic injury reveal oesophageal damage. This leads to subsequent oesophageal stricture in 10-15%. Mortality is less than 10% in unselected groups.

Accidents↗

The topographical and laminar organization of a commissural-associational entorhino-entorhinal projection in the guinea pig.

A commissural-associational entorhino-entorhinal projection in the guinea pig was analyzed using anterograde and retrograde axonal tracing techniques. The projection originated in layer II and terminated in layer Ia of both the medial entorhinal area (MEA) and the lateral entorhinal area (LEA). A few cells in other layers, especially layer III, also contributed to the commissural system. The projection was largely homotopic with the exception of the most medial MEA, which projected ventrally like previously described projections from the para- and presubiculum to the superficial layers of the entorhinal area. The commissural fibers crossed the midline in the dorsal hippocampal commissure. The antero-posterior position of the fibers within the commissure reflected the ventrodorsal position of their origin in the entorhinal area.

Animals↗