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Biomedical subjects

K Zuppinger

Publications and source records attributed to K Zuppinger.

At least 19 recordsLinked to original sources
PubMed1994

[The abused and neglected child in Switzerland].

Pediatricians form part of children's and young people's most important extra-familial relations. They are thus especially well placed: first, to discover abuse of any kind, and second to put in motion the first years of measures of assistance for the children and their families. The first years of life are decisive for effective prevention of abuse and neglect, and for the development of a healthy personality. In this part of life, pediatricians are virtually the only "social outposts". Nevertheless, in Swiss pediatrics the concept of child protection is still in the initial stages. While we should warmly welcome the fact this problem was at last the main theme of an annual meeting, it must be remembered that this was only the first time. For a long time now no one has doubted that in our, thus far socially privileged country, a frighteningly large number of children and adolescents are victims of abuse. Since the publication of the report "Mauvais traitements des enfants en Suisse" (1992) a representative questionnaire to parents has shown that in this country and now, as before, over a third of parents use corporal punishment on their children. It has been calculated that e.g. 21,800 babies aged between 0 and 2.5 years are beaten, 4800 of them even with implements. There are no data on psychological and sexual maltreatment. Despite this shocking incidence of abuse, only a total of 72 cases (6% of all recorded cases) were reported over one year by pediatric practitioners in the "1989 prospective study". We cannot accept that this reflects a lack of social concern. Many other shortcomings appear to be involved: lack of briefing on the problems of child abuse during medical training, post-graduate and continuing studies, inadequate arrangements for interdisciplinary work, discouragement and early delegation to pseudo-experts, distrust of the efficacy of available aids (but sometimes overestimation of one's own possibilities) and last but not least, a still highly idealized image of the family which prompts one to reject the possibility of abuse. The Swiss Pediatric Society is urgently called upon to focus closer attention on this subject, and in so doing to take advantage of the increasingly widespread concept that child abuse must be regarded as resulting from a disturbance of the child's social network. Here the pediatrician can find an effective, decisive and also--above all--preventive role.

Adolescent↗
PubMed1993

Persistent müllerian duct syndrome: a case report.

The persistent Müllerian duct syndrome is a rare disorder of sexual development. We report on a 4 month-old male who presented with a left-sided inguinal hernia and undescended testes. During the repair of the hernia 2 testes, 1 fallopian tube and an uterus were observed. The clinically suspected diagnosis of hernia uteri syndrome was confirmed by laboratory investigations. At the age of 18 months laparotomy was performed and the 2 fused gonades were descended into the right scrotum.

Chronic Disease↗
PubMed1991

Increasing incidence of hypoglycemic coma in children with IDDM.

OBJECTIVE: To examine the incidence of hypoglycemic coma in children with insulin-dependent diabetes mellitus (IDDM) over 8 yr from 1981 to 1988 and to investigate the importance of residual beta-cell function of HbA1 levels and other variables as risk factors for hypoglycemic coma. RESEARCH DESIGN AND METHODS: The study consisted of 155 children with IDDM aged less than 16 yr at study entry. Mean age at onset of diabetes was 7.9 yr (range 1.1-15.6 yr). We made a prospective assessment of hypoglycemic coma episodes, with a standardized questionnaire, over a total observation time of 816.6 person-yr. Three monthly clinical and laboratory examinations, which included determinations of C-peptide and HbA1 levels, were conducted. We compared children with hypoglycemic coma (cases) with children without hypoglycemic coma (controls) in a case-control analysis matched for diabetes duration. Yearly incidence of hypoglycemic coma, calculated as the number of subjects having an attack in 1 yr divided by the cumulative number of person-years for that year, was measured. Univariate and multivariate odds ratios were calculated from logistic regression. RESULTS: Over the first 4 yr, the average yearly incidence was 4.4/100 person-yr compared with 7.4/100 person-yr during the later 4 yr (P less than 0.0001). This tendency was accompanied by intensification of insulin treatment with an increase in the mean number of daily injections and a decrease in mean HbA1 levels. In the case-control analysis, absent residual beta-cell function was the most important risk factor for hypoglycemic coma (adjusted odds ratio 7.8, 95% confidence intervals 2.0-31.2), followed by near-normal HbA1 levels (adjusted odds ratio 4.5, 95% confidence intervals 1.9-10.5). CONCLUSIONS: In this group of children, improvement of glycemic control apparently led to an increase in the incidence of severe hypoglycemia. In children with recurrent hypoglycemic coma and undetectable C-peptide levels, it may be safer to aim for somewhat less tight glycemic control.

Adolescent↗
PubMed1990

[Hypoglycemia in childhood diabetes].

Severe hypoglycemic episodes in diabetic children are a serious complication of present medical therapy. With the recent trend towards intensified insulin therapy, the incidence of severe hypoglycemia will probably increase. The pathophysiological mechanisms in the development of severe hypoglycemia are lack of modulation of plasma insulin levels, diminished or abolished glucagon release, delayed epinephrine release, and diminished glucose threshold for awareness of hypoglycemic symptoms, especially in well stabilized diabetics. The consequences of severe hypoglycemia are EEG changes, focal or generalized convulsions, (rarely) partial or generalized epilepsy, and disturbances of cognitive function probably due to neuronal damage. Some of the risk factors can easily be understood and are preventable. A highly increased risk factor is a low HbA1, and a complete lack of endogenous insulin secretion. However, in our experience human insulin is not an additional risk factor. Home blood glucose monitoring for determining the correct insulin dose and food supply is of great prophylactic importance. In the presence of coma in a diabetic child due to hypoglycemia, i.m. glucagon or i.v. glucose should be administered immediately in the correct dose. Following a severe hypoglycemic episode the glucose equilibration should be somewhat less strict. Regular education of the patient on risk factors, prevention and therapy of hypoglycemia is of great importance.

Blood Glucose↗
PubMed1990

Unsustained central sexual precocity in four girls.

Four girls who presented with breast enlargement at 4-5.8 years of age have been followed without specific therapy for up to 4 years. Three had normal CT brain scans, one had normal skull and sella x-rays. Stimulation of gonadotropins by LHRH was excessive in all but plasma estradiol levels were only intermittently elevated. Initially, bone age was advanced and height velocity was increased in three of the four. Ultrasound visualized an enlarged uterus in two and waxing and waning ovarian cysts in all. The clinical course was characterized by persistence of physical signs over at least 3.4 years in one patient, fluctuation in another, and marked regression in two. We propose that some patients with central precocious puberty may spontaneously have a nonprogressive course which has to be considered when evaluating the efficiency of drugs interfering with puberty.

Age Factors↗
PubMed1990

[Basal bolus therapy in adolescent diabetic patients].

Between April 1986 and December 1987 30 adolescent patients with type 1 diabetes were changed from a conventional twice daily insulin regimen to the basal-bolus system, using pen-injectors. Actually, 26 patients are still using the new system. A comparison was made over a three-year period with a group of 26 patients on conventional therapy matched for age, sex and diabetes duration. A questionnaire was sent to the pen-injectors for subjective evaluation of the new system. The insulin dose remained unchanged. The incidence of hypoglycemic coma in the control group (4.3 per year/26 patients) was similar to the one in the pen-injector group prior to installation of the new system (4.0 per year/26 patients) and increased, but not significantly, on the new system (8.9 per year/26 patients). In both groups, the relative body weight increased significantly, the increase being greater in the pen-injectors (p = 0.001) than in the controls (p = 0.042); however, the difference of weight gain between the two groups was not significant. Fasting plasma cholesterol and triglycerides did not change. Glycosylated hemoglobin (Hb-A1 corrected for Hb-F) dropped significantly in the pen-injectors three months after installation of the new system (p = 0.026), but reached the preceding level already after six months. In the controls, the Hb-A1 remained constant over the three years. Greater flexibility in lifestyle, easier handling and better subjective diabetes control were the main advantages mentioned by the patients on the new system. Negative statements were the necessity for multiple injections, the high frequency of blood glucose control and strongly increased problems with weight control.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1989

[Hypoglycemia in children with diabetes treated with human or porcine insulin].

If hypoglycemia unawareness in diabetes is related to human insulin, its use would mean an increased risk of unconscious hypoglycemia. In a prospective study in 59 children treated from onset of diabetes by either human or porcine insulin of equal purity for a mean observation period of more than 3 years, no significant difference in the incidence of hypoglycemic coma was detected: 9/29 (31%) of the children treated by human insulin compared to 8/30 (27%) of those treated by porcine insulin had 1 or more severe hypoglycemic episodes. At the time of the first coma there was no significant difference in age, duration of diabetes, insulin dose, or HbA1 between the groups. Thus, human insulin is not considered to be an additional risk factor for the development of hypoglycemic coma in diabetic children.

Adolescent↗
PubMed1989

Increased prevalence of fetal haemoglobin in type 1 (insulin-dependent) diabetes mellitus.

Fetal haemoglobin levels were measured in 106 patients with Type 1 (insulin-dependent) diabetes mellitus during a period of two to three years. In 15 patients (14.1%) increased fetal haemoglobin levels (greater than 0.5%), determined by high pressure liquid chromatography, were found in contrast to 3% in a healthy control group (n: 100) of equal age distribution. In children aged over 6 years, elevated fetal haemoglobin levels were measured in 13 diabetic patients (13.3%) in contrast to none of the control group. There was no correlation between fetal haemoglobin levels and duration of diabetes, diabetic control (glycated haemoglobin) and dosage of insulin (U.kg-1, day-1). The 15 patients had a younger mean age at onset of diabetes (5.6 years) than a sex and age matched control group of diabetic patients without increased fetal haemoglobin levels (7.4 years, p less than 0.05). Longitudinal assessment revealed a significant decline of fetal haemoglobin levels with age (p less than 0.005) but a further increase in fetal haemoglobin levels were found in adolescent patients (n: 2). These data indicate a possible effect of insulin-treatment on delaying transition from fetal to adult haemoglobin synthesis or on reactivation of fetal haemoglobin production.

Adolescent↗
PubMed1988

[Disease course in 20 patients with an early diagnosis of phenylketonuria and hyperphenylalaninemia].

Twenty patients with PKU or hyperphenylalaninemia at ages 0.1 to 15.6 years (median age 6.2 years) were studied prospectively. In all children the condition had been diagnosed when they were neonates on the basis of an abnormal Guthrie test. To maintain plasma phenylalanine levels between 0.2-0.5 mM, dietary restriction of phenylalanine to 20-80 mg/kg daily (median 40 mg/kg) was necessary in 14 children. In children above 8 years, however, these plasma levels were frequently exceeded. In 6 children plasma phenylalanine levels were higher than normal diet. Height, weight and head circumference were within normal range in all patients at all ages. Determinations of DQ/IQ were done at 2, 4, 6 and 8 years of age and revealed values between 90-120 with a median of 102 in the 14 patients who were tested. Only 1 patient had IQ levels between 75-85 and attended special school. Nine other patients were in grade school performing averagely or above. This study confirms that early treatment and long-term follow-up of patients with PKU yield good results. Unsolved problems include duration of dietary treatment and the management of pregnancy in women with PKU.

Adolescent↗
PubMed1988

Intermittent microalbuminuria in children with type 1 diabetes mellitus without clinical evidence of nephropathy.

Microalbuminura (MA) was determined in 127 children and adolescents (age 3-21 years) with type 1 (insulin-dependent) diabetes mellitus. Patients with clinical evidence of long-term complications or macroproteinuria were excluded. Urinary albumin excretion was measured in a nocturnal 12-h collection and correlated with the albumin/creatinine ratio of a urine sample freshly voided on the morning immediately following the collection. The patients were divided into group A (n = 83, age less than 16 years, duration of diabetes 1-13 years, mean 4.4) and group B (n = 44, age greater than 16 years, duration of diabetes 1-19 years, mean 8.7) and compared with appropriate controls. MA above 15 micrograms/min was present in 11 of 83 (13.3%) patients in group A and in 7 of 44 (15.9%) in group B. In a repeat urine collection at least 3 months later elevated MA persisted in 1 of 11 (group A) and in 4 of 7 (group B) patients. There was no correlation between increased MA in a 12-h urine collection and the albumin/creatinine ratio in a subsequently voided urine sample. MA was not strictly dependent on age, sex, duration of diabetes, haemoglobin A1, mean arterial blood pressure, plasma creatinine, creatinine clearance or serum beta-2-microglobulin. Further systematic studies and careful follow up are necessary to appraise whether intermittent MA is indeed an early manifestation of incipient kidney disease in children with type 1 diabetes.

Adolescent↗
PubMed1988

Comparison of fructosamine and glycated haemoglobin in children with type 1 (insulin-dependent) diabetes mellitus.

In six children (age: mean 8.4 years, range 2.2-12.6 years) with newly diagnosed Type 1 (insulin-dependent) diabetes mellitus, plasma fructosamine and glycated haemoglobin (HbA1) were compared in respect to their disappearance during the first month after diagnosis during well controlled glycaemia. The disappearance of the surplus plasma fructosamine and HbA1 was calculated applying exponential equations. The estimated half-lives of fructosamine (mean 57.2 days, range 40.7-77 days) and HbA1 (mean 59.7 days, range 43.3-82 days) were not significantly different, a finding which is left unexplained.

Child↗
PubMed1988

Antihypertensive and metabolic effects of ketanserin in diabetic patients with mild hypertension.

Ketanserin is a serotonin S2 receptor antagonist with antihypertensive activity. Its effects on blood pressure, glucose metabolism and serum lipids were assessed in 24 patients with diabetes mellitus and mild arterial hypertension in a double blind, placebo-controlled trial. Ketanserin in doses up to 80 mg daily caused a slight decrease of supine BP (from 159/97 +/- 19/11 to 153/90 +/- 20/9 mm Hg; NS/P less than 0.01) and upright BP (from 160/102 +/- 18/13 to 151/93 +/- 12/12 mm Hg; P less than 0.05/NS). However, these pressures did not differ significantly from the levels observed in the placebo group. Supine and upright heart rate, body weight, plasma sodium and potassium, serum creatinine, glucose, C-peptide, glycosylated haemoglobin, serum cholesterol and triglycerides, their lipoprotein fractions, apolipoprotein A1, A2 and B concentrations and the responses of serum glucose and insulin to a standard oral glucose loading test did not change. These findings indicate that the selective S2 receptor antagonist ketanserin did not unfavourably influence glucose and lipid metabolism in diabetic patients with arterial hypertension.

Adult↗
PubMed1987

Comparison of human and porcine insulin therapies in children with newly diagnosed diabetes mellitus.

A multicenter, longitudinal study of children below the age of 16 years with newly diagnosed Type 1 (insulin-dependent) diabetes treated either with porcine monocomponent insulin (n = 26) or semisynthetic human monocomponent insulin (n = 26) was performed during the first 24 months after onset of diabetes. The two groups were carefully matched for age, duration of disease symptoms, initial metabolic values, islet cell antibodies and HLA-DR antigens. During the 24-month observation period there was no significant difference between the two groups in respect to the clinical course, insulin dosage, HbA1 and residual B-cell activity. No child in either group had a real remission without necessitating insulin therapy. The prevalence of insulin antibodies increased slowly and was 62% in the group treated by human insulin and 52% in the porcine insulin-treated group after 24 months. The titres were generally low and there was no statistical difference between the two groups in respect to insulin antibody formation.

Antibody Formation↗
PubMed1987

Neonatal diabetes mellitus: evaluation of pancreatic beta-cell function in two cases.

Two cases of neonatal diabetes mellitus, a transient form and a permanent form, are described. Comparing their clinical presentations and courses, we exclude the possibility of an early differential diagnosis based on clinical or laboratory data. We hypothesize that only repeated dynamic evaluations of pancreatic beta-cell function could be useful to differentiate the two forms.

Blood Glucose↗
PubMed1986

Familial growth hormone deficiency resulting from a 7.6 kb deletion within the growth hormone gene cluster.

We report on two sibs with familial isolated growth hormone deficiency (IGHD) resulting from homozygosity for a 7.6 kb deletion within the growth hormone gene cluster. The deletion not only affects the structural gene for growth hormone (GH-N) but also alters sequences adjacent to the chorionic somatomammotropin-like (CS-L) gene. In contrast to previously reported cases with IGHD type IA, our two patients responded well to growth hormone substitution and formation of blocking antibodies did not occur.

Chromosome Deletion↗
PubMed1986

Feminization in a galactosemic girl in the presence of hypergonadotropic hypogonadism.

A galactosemic girl has been followed in our clinic since her 8th day of life when the diagnosis of transferase-deficiency galactosemia was made until her present age of 21 years. Although she presented with direct hyperbilirubinemia and severe liver dysfunction, her subsequent somatic and intellectual development under a strict galactose-free diet was normal. Liver function normalized. Her pubertal stage was Tanner B II and PH II at age 11.7 years and progressed normally to B V and PH V, but menarche did not occur. At age 16.8 years, low estradiol (51 pmol/L) and high gonadotropin levels (LH 44 U/L; FSH 43 U/L) were measured. Severely hypoplastic ovaries with a streak-like aspect were seen on laparoscopy. Sex chromatin was positive. Follow-up studies over the next 4 years confirmed elevated LH and FSH levels with an excessive response to stimulation with LHRH and persistently low estradiol levels (41 to 117 pmol/L). After an intravenous load of 200 mg DHEA-S, plasma estrone levels rose 1.8-fold and estradiol 11.4-fold above basal, demonstrating prompt conversion of androgens to estrogens. For over one year, the patient has been treated with the synthetic progestin norethisterone (15 mg per day for 10 days in a row every month) which has resulted in regular menstrual bleedings.

Adult↗