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Biomedical subjects

H Wendler

Publications and source records attributed to H Wendler.

At least 19 recordsLinked to original sources
PubMed1990

Retrospective diagnosis of Jeune's syndrome in two patients with chronic renal failure.

Two patients are reported who presented at the age of 12 1/2 years with advanced chronic renal failure. Clinical findings and renal histology were consistent with nephronophthisis. The retrospective diagnosis of Jeune's syndrome was possible in both. Cone-shaped phalangeal epiphyses of hands and feet and metaphyseal chondrodysplasia of femoral heads and necks were the prominent findings on skeletal survey. One patient had a small bell-shaped thorax; in the other this aspect was present on reevaluation of neonatal X-rays. Our patients support the view that nephronophthisis is the principal cause of renal failure in Jeune's syndrome and that a wider awareness of this association could lead to detection of more so far unrecognized patients.

Asphyxia Neonatorum↗
PubMed1989

Bone disease of primary hyperoxaluria in infancy.

A patient with primary hyperoxaluria type I in infancy is reported. He had renal insufficiency, but urolithiasis was absent. Demonstration of diffuse nephrocalcinosis by renal ultrasound contributed to early diagnosis. Prolonged survival leads to extensive extrarenal oxalate deposition. Repeated skeletal surveys showed the development and the progression of severe hyperoxaluria-related bone disease. Translucent metaphyseal bands with sclerotic margins, wide areas of rarefaction at the ends of the long bones, and translucent rims around the epiphyses and the tarsal bones were signs of disordered bone growth. Bone density generally increased with time indicating progressive sclerosis due to oxalate deposition in the previously normal bone structure.

Bone Diseases, Metabolic↗
PubMed1985

[Menkes syndrome with excessive skeletal changes].

An infant was seen for multiple fractures at the age of 10 weeks. He developed marked cortical thickening of many bones, which raised the suspicion of a battered child syndrome. Unusual progression of bone thickening and hitherto undescribed excessive bone remodeling led to the diagnosis of Menkes'kinky hair disease, a disorder of the connective tissue caused by a decreased copper bioavailability, to which disease the infant finally succumbed.

Battered Child Syndrome↗
PubMed1983

[Sonographic diagnosis of nephrourologic diseases in infants and childhood].

Ultrasound is a harmless and renal function-independent screening procedure to detect malformations of the urinary tract, that need surgical intervention. Because of the paucity of symptoms concomitant with congenital malformations it is important to use this non-invasive exploration method frequently in the early course of the diagnostic investigations. Pathologic sonographic findings should be followed by radiologic exploration. In case of normal renal sonography in patients suffering from first urinary tract infection, further radiologic exploration of the upper urinary tract can be withheld, however ultrasound does not replace radiologic studies of the lower urinary tract. Complications after surgical procedures are readily depicted by sonography, thus avoiding the need for numerous post-operative radiologic study.

Child↗
PubMed1981

[Urinary testosterone excretion in boys in relation to other parameters of somatic maturity (a longitudinal study) (author's transl)].

The dependence of urinary testosterone excretion on age was studied in healthy male volunteers. Furthermore, it was determined monthly over 2 years in 20 boys aged 10 to 12 years. The values obtained for urinary testosterone were compared with the parameters of somatic growth like bone age, height, weight and testicular volume. The greatest intraindividual changes in testosterone excretion were found during puberty, and also a great interindividual difference in the onset of puberty. A relatively good correlation was obtained for the mean testosterone excretion and the bone age, on the one hand, and the testicular volume of a boy on the other hand.

Adolescent↗
PubMed1980

[The Carraro syndrome (author's transl)].

The report concerns a girl aged 9 1/2 years who was deaf and dumb and had marked shortening of the calves with deformities of the feet and bilateral, congenital hypoplasia of the tibiae. This syndrome was first described by Carraro in 1931, but there have been no further reports since then.

Abnormalities, Multiple↗
PubMed1978

["Late onset disease" (congenital german measles) (author's transl)].

The paper reports on a case of "late onset disease" (congenital German measles). Diagnostically significant for this disease in an infant appearing perfectly healthy when new-born, were mainly the findings of an interstitial pneumonia, hepatosplenomegaly, as well as findings indicating the presence of a meningoencephalitis and retinopathy. The diagnosis of this rare course of congenital German measles was finally established via the identification of specific IgM rubeola antibodies, of a raised hemagglutination inhibition titer, and of the German measles virus itself. An attempt to treat the interstitial pneumonia with cortisone preparations remained unsuccessful. The clinical and diagnostic problem complexes are discussed briefly.

Antibodies, Viral↗
PubMed1978

[Total aplasia of the diaphragm with hypoplasia of the lungs (author's transl)].

A case of congenital aplasia of the diaphragm with hypoplasia of both lungs is reported. By the complete absence of the diaphragm the liver could in the early stage of embryonic development expand around the heart into the thoracic cavity. This resulted in a growth arrest with consequently severe hypoplasia of both lungs, which were originally normally developed.

Autopsy↗
PubMed1978

[Pulmonary sequestration (author's transl)].

Pulmonary sequestration is a relatively rare condition in which a part of lung tissue has no or only a secondary connection with the tracheobronchial tree. Blood supply comes from the aorta in anomalous branches. Sequestrations may be intra- or extralobar. Gerle et al. include both types in the general term congenital bronchopulmonary foregut malformation. Two surgically confirmed observations of bilateral intralobar pulmonary sequestrations are reported. The clinical features are recurrent respiratory infections with cough, fever and purulent sputum. X-rays only show nonspecific alterations. Chronic changes in the lower lobes should draw attention to pulmonary sequestration. Thoracic aortography is the procedure of choice in establishing the diagnosis and should always be carried out preoperatively. Segmental resection or lobectomy is the indicated treatment.

Adolescent↗
PubMed1976

[Hereditary symphalangism: observations on this syndrome in a newborn infant and his mother (author's transl)].

Symphalangism is the partial or complete bony fusion of phalanges in the fingers and toes due to an autosomal dominant gene, with inherited failure of development of one or more interphalangeal joints. Two patients--a newborn baby and his mother--both revealing this rare malformation, are presented in this paper and the clinical and radiological features are described and discussed.

Adult↗
PubMed1976

[Differential diagnosis and course of the idiopathic juvenile osteoporosis (author's transl)].

A case of a twelve year old boy with idiopathic juvenile osteoporosis is reported. The disease is characterized by onset in the prebubertal or pubertal age. The initial complaint is deep pain of the extremities, followed by a decrease in growth and multiple fractures of the spine and lower extremities. Radiologically a severe generalized osteoporosis is shown. Diagnosis depends on the results of clinical and x-ray examination, bone histology studies and the ruling out other forms of osteoporosis, those due to other disease of bones, renal diseases and metabolic or endocrine disturbances. Spontaneous remissions usually follows 2-4 years of disease. A specific treatment is not known.

Age Factors↗
PubMed1975

[Therapy of secondary atelectasis by pulmonary lavage in full-term and premature infants (author's transl)].

Obstructive atelectasis can be successfully treated by means of pulmonary lavage. Atelectasis of this type may result from the formation of mucous plugs in the bronchi during or after prolonged artificial ventilation, after thoracic surgery and after vigorous suction. The pathophysiological alterations taking place during routine suction of an intubated and ventilated infant are discussed. The technique of pulmonary lavage with lukewarm physiological saline through an endotracheal tube is demonstrated in 2 newborn infants, in whom atelectasis occurred as a complication of long-term ventilation.

Female↗
PubMed1975

[Diagnostic problems in massive adrenal haemorrhage in the newborn (author's transl)].

Massive adrenal haemorrhage in the newborn frequently presents as an abdominal mass and is difficult to diagnose when this is the sole clinical feature. Surgery is indicated for the diagnosis and treatment of those cases in which a malignant tumour cannot be ruled out from the X-ray appearance with certainty. 3 cases are discussed in respect to clinical signs and the differential diagnosis of adrenal calcification in infancy is briefly mentioned in this connexion.

Adrenal Gland Diseases↗