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C Rudin

Publications and source records attributed to C Rudin.

62 records · Page 4Linked to original sources

Absence of the enteric nervous system in the newborn: presentation of three patients and review of the literature.

Three patients suffering from an absence of the enteric nervous system are reported. Two sisters presented with severe vomiting shortly after birth and dilatation of the intestine proximal to a stenosis. There was an absence of the enteric nervous system throughout the entire length of the intestine distal to the duodenum. A boy presenting an ileus was found to suffer from an aganglionosis of the entire colon. There was also an absence of neuronal bodies and nerve fibers in the small intestine. The final diagnosis was made by histochemical and immunocytochemical stains for acetylcholinesterase, lactate hydrogenase, neuron-specific enolase, protein S-100, and substance P. In the literature, 13 other patients have been reported. On the basis of differences of symptoms, incidence, sex ratio, genetics, and, presumably, pathogenesis between absence of the enteric nervous system and aganglionosis, it is assumed that the two diseases are separate entities.

Acetylcholinesterase↗

Zuelzer-Wilson's syndrome and absence of the enteric nervous system. Two rare forms of anomalies of the enteric nervous system with identical clinical symptoms.

47 children have been treated in Basel for aganglionosis (Hirschsprung's disease) during the last 25 years. Six children presented severe vomiting and an ileus as leading symptoms instead of chronic constipation, the classical leading symptom of Hirschsprung's disease. Clinical, radiological and intraoperative findings were virtually identical in these 6 patients. However, enzyme histochemical and immunocytochemical investigations disclosed an aganglionosis of the entire colon (Zuelzer-Wilson's disease) in 3 patients, absence of the enteric nervous system in the small and large intestine in 2 patients, and a combination of both in 1 patient. In contrast to children suffering from aganglionosis of the entire colon, the chance of survival for patients with an absence of the enteric nervous system is extremely small. It is therefore necessary in presence of severe vomiting and an ileus to take intraoperative biopsies from the large and the small intestine. The precise diagnosis can be made only by using enzyme histochemical and immunocytochemical techniques.

Cholinergic Fibers↗

[Children of drug-dependent mothers--personal experiences and review of the literature].

The increasing incidence of drug abuse in central Europe leads to a growing number of children with intrauterine drug exposure. During pregnancy various risks affect these children. Postnatally severe symptoms of withdrawal are observed frequently. The psychosocial development is endangered. Between 1986 and 1990 30 drug dependent women attended the obstetric department of the University Hospital Basel during pregnancy. The data of 21 children born to mothers drug dependent at birth were evaluated. 11 children were born in 1990. 12 mothers received Methadon, 10 were polydrug abusers. Positive serology was found for hepatitis in 18, for HIV-infection in 9 women. 6 babies were premature, 8 were small for gestational age. 20 newborns presented withdrawal symptoms which required a mean hospitalization time of 45 days. Tonic and/or clonic seizures were observed in 6 neonates. In 9 other children some symptoms were suspicious of seizures. EEG recordings were pathological in 10 of 12 infants. For the control of withdrawal symptoms 16 children received pharmacological treatment for a mean duration of 61 days. Neurodevelopmental examinations were performed in 14 children at an average age of 22 months. Neurological findings were pathological in 2 children. 3 had mild, 2 had severe mental retardation, 5 presented with behavioural disturbances. Our data are compared with those of the literature. Therapeutic proposals for children of drug dependent mothers are presented. The impact of methadon programs on the exposed children is discussed.

Dose-Response Relationship, Drug↗

[How often can the clinical diagnosis of exanthema subitum be confirmed with the HHV-6 serology test].

Three years ago a newly recognized human herpes virus (HHV-6) was identified to be the causative agent for exanthem subitum (roseola infantum). So far this diagnosis was suggested chiefly by excluding other infections and the presence of a typical course of the disease. To find out how often the clinical diagnosis can be confirmed serologically, we asked five pediatricians in private practice as well as residents of the out-patient and infant departments of our hospital to obtain paired heparinized blood samples of any child suspicious for exanthem subitum. 36 children with clinically suspected exanthem subitum and a mean age of 14.2 (5-71) months were included in this prospective study. Indirect immunofluorescence was used to detect IgG and IgM antibodies to HHV-6. In 22 children (61%) the clinical diagnosis was confirmed serologically. IgM antibody was found in only 16 of these 22 children. In 8 patients the results were ambiguous. Three had in the same time a doubtful seroconversion to other viruses of the herpes group (HSV twice; EBV once), in four children there was an insufficient rise in IgG antibodies without presence of an IgM response, and once we found a very high HHV-6 specific antibody titre (greater than 1:80) in both serum samples. In 6 children neither a seroconversion nor a rise in HHV-6 antibody titre were found. Retrospectively, only three of these children had a clinical course really typical for exanthem subitum. We conclude that in most cases the clinical diagnosis of exanthem subitum will be confirmed by serological examination.(ABSTRACT TRUNCATED AT 250 WORDS)

Antibodies, Viral↗

Non calcific vasculopathy in the basal ganglia.

Stripes of high echogenicity in the basal ganglia of infants have been rarely observed on cerebral ultrasound. These lesions, which appear to be due to a non calcific vasculopathy, are caused, in the majority of cases, by congenital infections and trisomy 13. We describe four infants with the characteristic ultrasonic appearance of this vasculopathy. Two children had cytomegalovirus (CMV) infection, one a human immunodeficiency virus (HIV) infection. The fourth child who presented a malformation of the skull and eye anomalies had no definitive diagnosis. Of the three surviving children one was developing normally. One had mild and one severe psychomotor retardation.

Basal Ganglia↗

[Premature labor in HIV infected women. Swiss "HIV and Pregnancy" Study Group].

In the Swiss Study "HIV and pregnancy" we observed 153 singleton pregnancies of HIV-positive women. 23 (15%) of those ended with a premature delivery. For drug addicts (n = 100), the incidence of prematurity, 20%, significantly higher than in those free of drugs (n = 53) with 5.6%. The most frequent cause of prematurity was premature labor or rupture of the membranes (n = 13), followed by maternal illness (n = 8) and fetal complications (n = 2). Women with premature delivery tended to have lower CD4 cell counts than those with term delivery (29.4% vs 12.0% with < 200 CD4 cells/microliters). Low CD4 cell counts and drug consumption are two independent but cumulative risks for severe infections. 16 of the 153 women (12 with, 4 without drug consumption) had severe infections during pregnancy; in 4 cases (25%), this led to prematurity. The most common infection was pneumonia (14/16), further one case of pyelonephritis and one of cerebral toxoplasmosis. Two of these 16 infants (12.5%) were HIV-positive. We could not confirm a relationship between prematurity and vertical HIV transmission. Of the HIV-classified children, 3/18 (16.7%) premature infants and 16/74 (21.6%) term infants were infected.

AIDS-Related Opportunistic Infections↗