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Biomedical subjects

D Claus

Publications and source records attributed to D Claus.

At least 163 records · Page 9Linked to original sources

[Congenital dilatation of the common bile duct. Apropos of l6 cases].

Congenital cystic dilatation of the common bile duct, also known as choledochal cyst, is an uncommon malformation, observed mainly in asiatic children, with a 3:1 female--male ratio. The entity is classified into 4 different types according to the anatomy of the malformation, which effects or not the intrahepatic biliary tree. The authors review theirs experience with 16 consecutive patients with choledochal cyst operated between 1971 and 1985. In 12 children (mean age: 5,8 years, 11 girls) without previous surgery, preoperative diagnosis was made with abdominal ultrasonography and, in some cases, endoscopic retrograde cholangio-wirsungography. Peroperative cholangiography demonstrated a common pancreatico-biliary channel associated with high choledochal amylase content in 6 cases. Surgical treatment consisted in cyst excision with hepatico-jejunostomy in those 12 patients. We also discuss the surgical treatment of 4 previously operated patients with postoperative cholangitis or biliary carcinoma. An anomalous pancreatico-biliary ductal junction (long common channel allowing free pancreatico-biliary reflux) is usually observed in these patients; it is considered to play a role in the etiology of congenital choledochal cysts. Appropriate surgical treatment consists in cyst excision with hepatico-jejunostomy as biliary drainage, in order to eliminate bile stasis, to remove the mechanism which allows continuous pancreatico-biliary reflux and to prevent the occurrence of bile duct carcinoma.

Adolescent↗

Congenital fibrosarcoma. Preoperative chemotherapy and conservative surgery.

Three cases of congenital fibrosarcoma are reported. The use of preoperative chemotherapy, a VAC regimen, allowed conservative surgery in two of them. The three children are well, with no evidence of disease and without sequelae after completion of postoperative chemotherapy.

Antineoplastic Combined Chemotherapy Protocols↗

The challenge of upper airway obstruction in pediatric intensive care.

Over a 5-year period, 75 patients were admitted to a pediatric intensive care unit because of acute upper airway obstruction (AUAO). Congenital abnormalities were demonstrated in 25 children; 9 of them died secondary to severe combined malformations or major central nervous system dysfunction. Acquired AUAOs were seen in 50 patients; 1 died in brain death secondary to anoxia. Obstructions were observed at all levels of the airways from the nose to the carina. In 28% of the patients, AUAO had remained undiagnosed until admission for complications such as acute respiratory failure, cardiac arrest, coma, seizures. With early diagnosis and prompt release of the obstruction the prognosis is good unless severe congenital abnormalities or major central nervous system damage are associated.

Airway Obstruction↗

The relationship between long latency responses and height.

Long latency responses to electrical stimulation of nerve trunks in upper and lower extremities were studied in 83 healthy subjects. The latency differences between the late responses, M2, and the segmental reflexes, M1, on the hand correlated with the height, confirming a transcortical reflex loop. Corresponding to the possible polysynaptic spinal origin of the late reflex response on the lower leg, there was no correlation with height. Therefore, it is not possible to calculate the intraspinal conduction time from long latency reflex examinations.

Adult↗

Long latency muscle responses in cerebellar diseases.

Long latency reflexes were measured from the hand muscles of 27 patients suffering from different cerebellar diseases (12 diffuse cerebellar atrophies, 7 cerebellar hemispheric infarcts, 8 Friedreich's disease) and from 45 controls after electrical stimulus of the median nerve at the wrist. The M3 response (latency about 70 ms) was increased in about 50% of cerebellar atrophy cases and occasionally (10 of 12 cases) separated from the M2 response (50 ms). M3 was sometimes (3/7) increased and the M2-3 complex was prolonged ipsilaterally in cases of cerebellar infarcts. In the cases of Friedreich's ataxia M2 was always lost uni or bilaterally because of the disturbance of afferent or efferent fibres. The latencies of the spinal reflex M1 and also of M2 were not always increased strongly enough to be clearly separated from the normal values.

Adult↗

Prevalence and treatment of silent gastro-oesophageal reflux in children with recurrent respiratory disorders.

Thirty-six infants and children presenting with recurrent respiratory disorders (RRD) as the sole clinical symptom including bronchial asthma (6), recurrent obstructive bronchitis with or without wheezing (18), chronic nocturnal cough (3), recurrent episodes of pneumonia (3), recurrent pharyngitis (3) and recurrent laryngitis (3) were investigated for associated gastro-oesophageal reflux (GER) by oesophagram, endoscopy and continuous 24 h pH monitoring of the distal oesophagus. The pH monitoring criteria were selected on the basis of a preliminary study comparing statistically measurements of 32 variables recorded in 15 patients who all had clinical, radiological and endoscopic evidence of GER and in 8 asymptomatic controls. Although patients with symptomatic GER differed significantly from the asymptomatic ones for 27 variables examined, 6 variables emerged as having the highest value for discrimination (overlap score 0-1). Among these, the Euler-Byrne index (number of reflux pH less than 4 + 4 times the number of reflux episodes of more than 5 min), the percentage of total reflux time and the number of reflux episodes 1 h post-cibal scored 0 (no overlap). GER was considered to be present when at least five of these six parameters were abnormal. The overall incidence of GER in children with RRD was 41% (15) when detected by oesophagram and 61% (22) when diagnosed by pH monitoring criteria. In the children with bronchial asthma or with recurrent laryngitis, the percentage of reflux time during sleep was about 40 times higher than in asymptomatic controls and 2 times higher than in those with symptomatic GER.(ABSTRACT TRUNCATED AT 250 WORDS)

Child↗

[Long loop reflexes--a clinically relevant method].

Late reflex potentials have been know for a long time. On the upper limb it has been proven that these potentials have a transcortical pathway. The electrical stimulation of nerve trunks is easily applicable in clinical practice and produces clear long-loop responses. The typical results can be reproduced for extrapyramidal, cerebellar and pyramidal lesions by this method. The long-loop reflex is sensitive to lesions in the course of the pyramidal tract.

Adult↗

[Differential diagnosis, pathogenesis and therapy of alcoholic polyneuropathy].

The most common forms of polyneuropathies are the alcoholic and diabetic polyneuropathies. They each constitute 1/3 of all polyneuropathies. The first symptoms shown by the alcoholic polyneuropathy are symmetric sensory disturbances with loss of tendon reflexes and of vibration sense in the peripheral segments of the lower extremities. At the beginning one almost always finds pressure pain in the calves. Important differential clues in diagnosis compared to the diabetic neuropathy, are the age at which the disease begins, the degree to which the autonomic nerve fibres and the cranial nerves are affected, as well as the form of manifestation. Pathogenetically, a direct toxic alcohol effect can above all be suspected in accordance with the typical electrodiagnostic findings with a neurogenic pattern in the EMG in the case of normal or slightly diminished conduction velocity, and in agreement with the morphological finding of an axonal degeneration in most of the biopsies. Possibly, in a small number of cases a vitamin deficiency or a malabsorption can play a causal role. The prognosis is good by complete abstinence from alcohol.

Alcoholism↗

[Fistulas of the 3rd and 4th branchial clefts].

The third and fourth branchial fistulas are exceptionally rare; both originate from the pyriform sinus, extend through the inferior constrictor of the pharynx and cross either the upper or the lower aspect of the superior laryngeal nerve, downwards and frontwards. Like the more common first and 2nd branchial fistulas, a cervical abscess is the usual consequence of these fistulas which should be treated by radical excision.

Branchioma↗

[Subacute osteomyelitis in young children. Study of 17 cases].

Subacute osteomyelitis is characterised by absence of symptoms of generalised infection and a favourable prognosis. In the last 15 years, the authors have observed 17 such cases in children, aged between 3 months and 4 years. Three were situated in the epiphysis and 14 in the metaphyseal region. On three occasions, a tarsal bone was involved and in five cases the lesion was in the spine. The radiological signs were very characteristic and biological changes were minimal. Exploration of the site of the infection was done on ten occasions, but only on two were organisms found. Treatment was based on antibiotics active against staphylococci and drugs inhibiting prostaglandins. Only one case was treated surgically. Healing occurred eventually in all cases.

Anti-Bacterial Agents↗

Thiamine deficiency and nervous system function disturbances.

Thiamine is important for oxidative metabolism, and B1 deficiency is thought to give rise to polyneuropathies. A group of male Wistar rats (n = 15) received a vitamin B1 deficient diet (group-a), and the pair fed control group (n = 20, group-b) received a normal diet with no vitamin deficiency. A second control group (group-c) was fed unrestrictedly with a standard diet (n = 19). All animals were examined for 25 weeks. The sensory nerve conduction velocity, the compound radicular, spinal and brain stem responses and the SEP were derived for tail and hind paw stimulation. The examination was repeated at 6-week intervals. There was no difference in nerve conduction between group-a and -b, but for both groups the conduction velocity was significantly slower than in group-c. The SEP latencies were significantly increased in group-a compared with group-b and also with group-c. The spinal and cerebral latencies were delayed in group-a. The diameters of myelinated nerve fibres were decreased in group-a compared with group-b, and in group-b compared with group-c. The results indicate that a specific polyneuropathy exists as a result of B1 deficiency, and that the sequelae of the lack of thiamine are pronounced in the CNS.

Afferent Pathways↗

Sjögren's syndrome in a child.

Sjögren's syndrome is a relatively uncommon condition in the paediatric age group. The youngest child reported thus far was a 5-year-old girl. This article reports the case of a 2-year-old girl admitted for recurrent infections of the respiratory tract with diffuse pulmonary interstitial infiltrations and a progressive swelling of the parotid glands. The clinical features and the results of laboratory investigations, including parotid and hepatic biopsies, chest X-rays and sialography helped to establish the diagnosis of Sjögren's syndrome. Clinical, immunological and genetic characteristics of Sjögren's syndrome are reviewed.

Biopsy↗

Ethanol and polyneuropathy.

Two groups of alcoholics (30 patients each)--identified by the MALT score--were examined. Clinical and laboratory investigations showed no connection between thiamine, riboflavin, or Vitamin B6 deficiency and development of the polyneuropathy. Neither the polyneuropathy nor the diminished sensory conduction velocity were related to malnutrition. The relation between the duration of alcoholism and symptoms of polyneuropathy was highly significant in one group. The neurotoxicity of ethanol was confirmed in an experiment with rats.

Adult↗

[Liver transplantation in children].

Liver transplantation has become a clinical therapeutic modality for end stage liver diseases. The results achieved in children are better than in adults: in T.E. Starzl unique experience in Pittsburgh, USA, the survival rate at one and four years are 75 and 70% respectively. Complete rehabilitation of these children can nowadays be expected. Between March 1984 and June 1985, 8 children received an orthotopic liver transplantation at the University of Louvain Medical School in Brussels, Belgium; one child received two transplantations after acute and irreversible rejection of a first ABO incompatible graft. The indications were biliary atresia in five (polysplenia in one), biliary hypoplasia in one, alpha-1-antitrypsine deficiency in one and Crigler-Najjar syndrome type I in one. The age of the patients at the time of liver replacement was 12 to 18 months in four, 8 to 13 years in four. Six patients are alive after 17, 14, 12, 10, 3 and 3 months; the two youngest children deceased during the first postoperative month. The Kaplan-Meyer one year survival rate is 75%; all surviving children are in excellent clinical condition with a normal liver function. The 9 transplanted livers were harvested from multiorgan cerebral death donors with the exception of one neonate whose liver alone was removed; 4 were retrieved locally, the five others were offered by foreign hospitals through the organ procurement agencies (Eurotransplant, France-Transplant, U.K. Transplant). Due to appropriate logistics with air flight transportation of the harvesting team when indicated, the total ischaemia time was kept below 6 hours in every case. Two small children underwent a left lobe orthotopic transplantation after ex vivo right trisegmentectomy of the liver retrieved from an older donor with one long term survival. The indications for liver transplantation in children are end-stage liver diseases consisting of a) cholestatic diseases among which the most frequent is biliary atresia after unsuccessful Kasai procedure followed by familial cholestasis (Byler syndrome) and the paucity of the intrahepatic bile ducts of the syndromatic (Alagille syndrome) or non syndromatic type. b) the metabolic diseases resulting either in cirrhosis with liver failure (alpha-1-antitrypsin deficiency, Wilson disease, glycogen storage disease type I and IV, protoporphyria) or in extrahepatic complications of enzymatic deficiency of an otherwise normally functioning liver (Crigler-Najjar syndrome type I, familial hypercholesterolemia and perhaps oxalosis). c) the hepatocellular diseases either chronic with cirrhosis of various origin or acute, eg. toxic hepatitis.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗

[Relation between long loop reflex findings and the topography of cerebral infarcts].

27 patients with demarcated cerebral infarctions were examined in the computed tomograms. The long loop-reflexes were elicited by electrical stimulation of the median nerves. The localization of substance defects was clearly related to the loss of the late M2-potentials. This result points out a transcortical reflex loop through medial lemniscus, ventral posterolateral nucleus of the thalamus, dorsal limb of the internal capsule, corona radiata and somatosensory cortex.

Adolescent↗

[Neurological complications of chronic alcoholism].

Almost all the patients admitted over a six-month period and treated in the neurological and psychiatric University departments at Lübeck because of alcoholism and its sequelae were submitted to a standardized neuropsychiatric investigation. The diagnosis was confirmed by the Münchner Alkoholismustest (MALT). Alcoholic polyneuropathy was the most frequent neurological sequel, affecting 30% of the 153 patients, followed by delirium tremens with an incidence of 21.6% and by alcohol-induced seizures (20.9%). Wernicke's encephalopathy was diagnosed in 3 cases (2%) and alcoholic cerebellar atrophy in 2 cases (1.2%). Hepatopathy was established in 43.8% of all cases. Neither the duration of alcoholism, the preferred kind of liquor, the patient's social class nor nutritional status showed any significant correlation to the appearance of neurological complications. Delirium tremens and alcohol-induced seizures appeared most frequently in cases with symptoms of diffuse brain damage, as measured by the incidence and severity of organic psychoses. The characteristic features of the various secondary diseases are pointed out and their pathogenetic connections are discussed in the light of the clinical findings.

Adolescent↗