Search PubMed⌕ Search

Biomedical subjects

I Gassner

Publications and source records attributed to I Gassner.

54 records · Page 3Linked to original sources

[Aberrant course of the left pulmonary artery--a contribution to a rare disease picture].

An anomalous left pulmonary artery obstructs the right main bronchus and/or the trachea. Depending on the severity of the obstruction, symptoms range from a more or less disturbing stridor over attacks of shortness of breath to a life-threatening or even lethal apnoea. An 18-month-old girl suffering from a mild form of this anomaly is presented. The diagnostic and therapeutic possibilities are discussed. After exact clarification of the characteristics of the pulmonary sling, exclusion of associated aberrations, and considering good experience with a former case a conservative approach is recommended if the patient is not substantially impaired.

Bronchial Diseases↗

Early surgery in the management of pediatric blunt renal trauma.

Of 40 children with major blunt renal injuries managed surgically 30 (75 per cent) could be followed up to 13 years postoperatively (mean 4.5 years). Measurements of renal size on sequential excretory urograms demonstrated normal growth of the traumatized kidney in all but 1 child. No hypertrophy of the contralateral kidney was noted in any child. Kidney length correlated with renal plasma flow calculated by sequential scintiscans (r equals 0.77). Hypertension was noted in 3 patients. Split renal vein renin studies and angiography showed that hypertension was caused by renal artery stenosis in 1 patient. Although we cannot draw any firm conclusions with regard to the optimal management of major blunt renal injuries, we present precise data that are valuable in comparison to other series of patients managed conservatively or surgically.

Adolescent↗

[Tuberculous otitis media and mastoiditis in 2 children].

One hundred years ago Robert Koch discovered tuberculous bacilli in tuberculous tissue. Since then the tuberculous disease has shown decreasing frequency, due to the specific antibiotics (Streptomycin since 1944) and to the BCG-Vaccination (Calmette and Guerin, 1921; vaccinations since 1948). This leads to difficulties in diagnosing the disease, especially in extrapulmonary forms (present in about 1:7 cases). One must consider also that about 40% of new diseases occur in the first 5 years of life and 80% in the first 14 years [9]. We report on two children with tuberculous otitis/mastoiditis in whom the lung disease was subsequently discovered. In one of the cases the mother died of tuberculous meningitis a few days after the specific otitis had been diagnosed in the child.

Age Factors↗

[The use of midodrin in the treatment of the orthostatic syndrome (author's transl)].

Midodrin, a new alpha-mimetic drug, has been studied with respect to its therapeutic effectiveness in children suffering from orthostatic hypotension. The study was carried out as a double blind trial against placebo. A statistically significant improvement was found only in relation to the following symptoms: "tendency to collapse", "dizziness", "headache", "change of colour". Blood pressure- and ECG alterations within the framework of the Schellong orthostatic and functional test were not significantly different statistically. This might be due to the small number of patients examined, to a placebo-effect or perhaps due to the inconsistent findings inherent in this last test.

Child↗

[Mucocutaneous lymph node syndrome or infantile perlarteritis nodosa with extensive vascular involvement].

The authors report on a male infant displaying all the clinical features of the mucocutaneous lymph node syndrome (MLNS) as described by Kawasaki in 1967. In the same patient, diffuse dilatation of the whole arterial tree--as known in infantile periarteriitis nodosa but so far not shown in MLNS--could be demonstrated angiographically during the acute phase of the disease. As the vascular findings at postmortem examination in MLNS and periarteriitis nodosa were the same too, the authors believe that separation of these diseases is not justified.

Aneurysm↗

[The double-chambered right ventricle (author's transl)].

Report on two girls with an obstruction within the right ventricle beneath the infundibulum. A muscular band - probabaly the abnormally situated moderator band - emerges at right angles from the ventricular septum, crosses the lumen and ends at the anterior wall of the right ventricle, thus separating a high-pressure inflow chamber from a low-pressure out-flow chamber. The latter compartment is distinctly larger than the infundibular chamber in Fallot's tetralogy. In bt connecting the left ventricle with the hig-pressure compartment. In one patient an increase of severity of the obstruction could be documented; this child showed a subvalvular membranous aortic stenosis. The diagnosis has to be established by meticulous pressure recording between pulmonary and tricuspid valve and by dye injection into the right ventricle. Ventricular septum defect (in 80%), subvalvular aortic stenosis (in about 10%), pulmonary valvular and peripheric stenosis and open Ductus Botalli are the most important accompanying lesions. The malformation - not diagnosed prior to surgery - causes serious trouble during operation. The investigator thus has to be aware of this condition in every pulmonary valvular ahd subvalvular stenosis especially when accompanied by a ventricular septum defect.

Cardiac Catheterization↗

Familial occurrence of syngnathia congenita syndrome.

A case of familial occurrence of syngnathia congenita in mother and child is described; this syndrome has been seen only sporadically until now. Due to the distinct appearance of the intraoral chords--they were situated on both sides between the ridges of the jaw and also between the left half of the bifid uvula and the retrolingual region--a relationship to the autosomal-dominantly transmitted Cleft Palate Lateral Synechiae Syndrome (CPLS-Syndrome) is suspected. Complete penetrance with different expressivity may explain the different clinical manifestations of the cases seen up to now and the absence of bifid uvula in the mother of our patient. The probable pathogenesis during embryological development is discussed.

Adult↗

[Familial occurrence of Elfin's face (Williams-Beurens Syndrome =wbs) and supravalvular aortic stenosis (= svas) (author's transl)].

Three siblings out of two families -- the mothers being sisters -- have signs of WBS: a girl shows the complete picture of elfin's face syndrome; a boy who needed surgical treatment for severe supravalvular aortic stenosis, presents only with minimal signs of the peculiar facies like his mother. His brother has slight supravalvular aortic stenosis. The three children described show trivial peripheral pulmonary stenosis. It is known that SVAS and WBS may show a dominant mode of inheritance with variable expressivity. The purpose of this study was to find signs which would prove a dominant inheritance in this kinship. Dental malpositions and coarse upperlip with shallow philtrum where the only symptomes found in the patients and their mothers. If we accept this traits as markers for the syndrome than we could assume a dominant way of inheritance.

Abnormalities, Multiple↗

[Bird-headed-dwarfism. A case report (author's transl)].

The case history and symptoms of a girl aged 12 months are reported suffering from bird-headed-dwarfism or Seckel-Syndrom: low birth weight in spite of prolonged pregnancy, typical "bird-headed-face", mikrocephaly, dwarfism, dystrophy, mental-motoric retardation.

Abnormalities, Multiple↗

[Heroin-withdrawal-syndrome in the newborn (author's transl)].

A case of a heroin-withdrawal-syndrome is reported. Diagnostic, therapeutic and social aspects of this syndrome are discussed. Newborn infants delivered from mothers addicted to heroin often develop a deprivation syndrome. 40% of these children are small for date babies. Symptoms of deprivation begin after delivery or between the 1st and 4th week of life. Generally the onset of symptoms lies between the 1st and 3rd day of life with irritability, tremor, restlessness, and shrill cry. The degree of illness depends upon mother's last heroin intake. Treatment for drug addicts are tinctura opii (3--5--10 drops orally following 4 hours) and Chlorpromazine (2,2 mg/kg/25 hours orally or i. m.). The mortality rate increases when treatment is missing.

Electroencephalography↗

[Diagnosis of diastematomyelia using ultrasound].

During the last two years diastematomyelia was found sonographically in 4 patients and confirmed by myelography combined with CT-myelography. In one case an autopsy was done. Three times ultrasounddiagnosis was established during the newborn-period. Spinal sonography allows soonest diagnosis even in severely ill newborn and premature infants and provides a basis for further examination (M, CT M and MRI). The clinical and radiological features of diastematomyelia and the associated malformations are explained and the indications for spinal sonography in the newborn-period are stressed.

Child, Preschool↗

[Pulmonary sling or ring-sling complex? respiratory problems--diagnosis, therapy, prognosis].

PS is a rare congenital vascular anomaly in which the left pulmonary artery originates from the right pulmonary artery and courses between trachea and esophagus to the hilus of the left lung causing compression of the right mainstem bronchus and trachea. In about half of all cases this vessel anomaly is associated with tracheo-bronchial and/or cardiovascular malformations. We present 6 patients with PS concentrating on the accompanying malformations of the tracheobronchial tree. All patients had the typical symptoms of wheezing and stridor already in early infancy. 3 patients showed concomitant tracheobronchial anomalies. 2 patients had additional cardiovascular deformities. In 3 of the patients the positive clinical course allowed conservative therapy. In the case of 2 further patients the respiratory problems demanded surgery, one of this patients died. A further patient died after palliative surgery of cardiac anomaly.

Adolescent↗

Twenty-three years of follow-up in patients with total colonic aganglionosis.

The main purpose of our study was to evaluate the outcome of patients with total colonic aganglionosis diagnosed and treated in our hospital. Seven of our twelve patients died within 6 month after birth due to infectious complications or underlying other diseases. 5 patients are alive after subtotal colectomy and ileo-rectal anastomosis, and were investigated for late complications, social integration, stool behavior, local status and laboratory parameters 12 and 23 years after surgery. All patients developed almost normally and were socially and professionally integrated. Although all had an accelerated passage of stools, no signs of malnutrition were found. One patient developed a recto-sacral fistulae and sacral osteomyelitis eight years after surgery and needed an ileostomy. After ileo-rectostomy all patients were continent with a grown rectal stump, had no strictures and a normal appearance of the mucosa.

Adolescent↗

[Pulmonary lymphangiectasis with spontaneous chylothorax in Noonan syndrome].

We report a case of Noonan syndrome associated with pulmonary stenosis and major lymphedema of the lower extremities. At the age of 15 yr spontaneous chylothorax with increasing dyspnea occurred> Chest-x-ray demonstrated increased interstitial markings restricted to the right lower lobe representing pulmonary lymphangiectasia. The chylothorax did not respond to repeated thoracocentesis and medium-chain-triglyceride diet. When a chest tube was inserted and total parenteral nutrition was supplied, the chylous effusion decreased within 32 days. The patient is still on diet and asymptomatic effusion remained during 12 months follow up. In conclusion, pulmonary lympgangiectasia should be considered in patients with Noonan syndrome and an abnormal interstitial pulmonary pattern similar to pulmonary congestion (without any hemodynamic abnormalities). In case of pleural effusion, chylothorax should be considered.

Adolescent↗

US of the spinal cord in newborns: spectrum of normal findings, variants, congenital anomalies, and acquired diseases.

Ultrasonography (US) of the spinal cord is performed in newborns with signs of spinal disease (cutaneous lesions of the back, deformities of the spinal column, neurologic disturbances, suspected spinal cord injury due to traumatic birth, and syndromes with associated spinal cord compression). The examination is performed with high-frequency linear- and curved-array transducers in the sagittal and axial planes from the craniocervical junction to the sacrum. Normal variants such as transient dilatation of the central canal and ventriculus terminalis can be demonstrated with US. US allows detection of congenital malformations, such as myelocele or myelomeningocele, spinal lipoma, dorsal dermal sinus, tight filum terminale syndrome, diastematomyelia, terminal myelocystocele, lateral meningocele, caudal regression syndrome, and hydromyelia or syringomyelia. Acquired intraspinal diseases following birth trauma and transient alterations after lumbar puncture can also be detected with US. US can demonstrate the entire spectrum of intraspinal anatomy and pathologic conditions with high geometric resolution. Therefore, US should be considered the initial imaging modality of choice for investigating the spinal cord in newborns.

Birth Injuries↗