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Biomedical subjects

H Kemperdick

Publications and source records attributed to H Kemperdick.

At least 19 recordsLinked to original sources
PubMed1994

Oto-palato-digital syndrome type II in two unrelated boys.

We report on two boys with oto-palato-digital syndrome type II, characterized by growth retardation, bowed long bones, missing or hypoplastic fibulae, sclerosis of the skull base and wavy, irregular clavicles and ribs. The facial appearance is distinctive due to prominent forehead, widely spaced eyes, antimongologid slant of palpebral fissures, flattened nasal bridge and retrogenia. The mother of one patient showed a mild manifestation of oto-palato-digital syndrome type II. Only about 20 cases of this rare X-linked disorder have been reported so far. The similarities and dissimilarities to oto-palato-digital syndrome type I are discussed.

Abnormalities, Multiple↗
PubMed1990

Neonatal radiological aspect of enchondromatosis (Ollier's disease).

A neonatal case of enchondromatosis is reported on, presenting with typical clinical features and radiological changes at birth. At two years of age, the enchondromata had considerably enlarged, and diagnosis was established by biopsy. The radiological aspect of enchondromatosis in the neonate is described and compared to its development in early childhood.

Enchondromatosis↗
PubMed1987

Prognostic significance of tumor volume in localized Ewing's sarcoma of bone in children and adolescents.

A total of 60 consecutive patients with localized Ewing's sarcoma of bone who were entered into the Cooperative Ewing's Sarcoma Study of the German Society of Pediatric Oncology from January 1981 until April 1985 were evaluable for tumor volume at diagnosis. The tumor volume was calculated from plain X-rays and CT scans as ellipsoidal or cylindrical depending on the tumor configuration and presence or absence of a soft tissue component. The 3-year disease-free survival rate according to Kaplan-Meier life table analysis was 78% for tumors with a volume less than 100 ml compared to 17% for tumors greater than or equal to 100 ml volume. These results were independent of the site of the tumor, though larger tumors were primarily located in central and proximal extremity sites. Maximal tumor extension was less precise than tumor volume in predicting prognosis. The ratio of tumor volume to body surface area, body length, or body weight did not increase the ability to separate prognostic groups compared to tumor volume. The better prognosis for patients following radical surgery seems to be in part due to a biased distribution of tumor volumes within local therapy groups, since more patients with smaller tumors had surgery for local control.

Adolescent↗
PubMed1986

[Skeletal age determination in children].

Determination of skeletal age in the growing patient is very important for various diagnostic questions. It is the most objective criterion of growth and development. Accordingly, it is necessary to be familiar with the most common methods used at present to estimate bone age in childhood. In newborns and infants skeletal age is usually determined from a lateral x-ray of the left lower leg, including knee and foot; after three month of age, an x-ray of the left hand is used. We present methods of assessing bone age from lower leg (v. Harnack) and from the left hand (Greulich-Pyle; Tanner-Whitehouse; de Roo and Schröder).

Age Determination by Skeleton↗
PubMed1984

[Pulmonary dysplasia in infancy. Pathogenesis, pneumologic course studies and therapy possibilities].

Some premature and mature newborns who require intermittent positive airway pressure (IPPV) and high oxygen concentrations for respiratory distress syndrome develop characteristic damage of bronchi and bronchioles termed broncho-pulmonary dysplasia (BPD). According to the radiographic findings the changes are categorized into four progressive stages. Stage 1 describes a radiographic feature with a pattern of fine, faint granularity as it is characteristic for the hyaline-membrane-syndrome. Stages 2 to 4 represent diffuse interstitial emphysema, a bubbly appearance of the lung, atelectasis and a progressive fibrosis. Electronmicroscopic investigations of bronchial imprints could demonstrate a rarefication of the cilia and a ciliary damage which took the form of compound cilia. In addition, a marked increase of goblet cells in the bronchial mucosa as well as a metaplasia of the epithelial cells was present. These findings may be a prerequisite for chronic infections, and perpetuate a cycle which may result in chronic obstructive airway disease. The significance of bronchial and bronchiolar injury in children with BPD is said to be due to IPPV, high inspiratory oxygen concentrations, high fluid intake, vitamin E deficiency or an increased intrapulmonary pressure secondary to a patent ductus arteriosus. When pulmonary mechanics were measured in a baby-body-plethysmograph a high pulmonary resistance and a low dynamic compliance occurred at the first investigation after IPPV or oxygen administration. On re-examination there was a strong tendency to normalisation of x-ray findings and pulmonary mechanics, depending upon the time which elapsed between ventilation and re-examination. Current therapy has to be symptomatic and may include secretolytics, glucocorticoids and bronchodilators. The preventive interventions have to take into consideration ventilation techniques, restrictions in O2 and fluid intake.

Bronchi↗
PubMed1983

Are bowing of long tubular bones and preaxial polydactyly signs of the Meckel syndrome?

We describe four cases with signs resembling those of Meckel syndrome. Two cases demonstrated postaxial polydactyly; one case, preaxial polydactyly; and one case, pre- and postaxial polydactyly. Since there is at least one other reported case with preaxial polydactyly, it may be a rare sign of the Meckel syndrome. In all four cases, various degrees of bowing of the long tubular bones were observed. Since at least two cases exhibited typical Meckel syndrome and since in a few further reported cases X-ray examination revealed bowing of long tubular bones, this sign is considered to be a further, hitherto not well recognized sign of the Meckel syndrome, and not grounds for delineation of a new syndrome. An extensive review of the literature revealed, that shortened and bowed extremities may be present in about one-sixth of all cases with Meckel syndrome.

Abnormalities, Multiple↗
PubMed1982

Thanatophoric dysplasia with cloverleaf-skull. Case report and review of the literature.

The radiologic and morphologic features of thanatophoric dysplasia with cloverleaf-skull could be demonstrated in a neonate who died from asphyxia shortly after birth. Occipital bone hemangiomatosis, a typical histomorphologic finding in cloverleaf-skull, is reported for the first time in a thanatophoric dwarf. Our investigations and the discussion of 26 observations of thanatophoric dysplasia with cloverleaf-skull reported since 1967 do not yet allow complete understanding of the pathogenetic relationship of both malformations. They serve, however, to elucidate some crucial unanswered questions which it is suggested should be studied in future observations of thanatophoric dysplasia with cloverleaf-skull.

Dwarfism↗
PubMed1981

The Weaver syndrome: a rare type of primordial overgrowth.

A boy with primordial overgrowth, macrocephaly, and anomalies of the face, nails, feet and skeleton is reported. Two cases in the literature- referred to as Weaver syndrome- exhibited nearly identical anomalies. All three cases were sporadic. Main symptoms of the Weaver syndrome are increased birth weight, early overgrowth, macrocephaly, accelerated osseous maturation, typical facies, hoarse, low pitched voice, hypertonia of muscles and mild developmental delay. Further symptoms are thin, deep-set nails, talipes equinovarus, widened distal femora, and some minor abnormalities. A second boy with primordial overgrowth and macrocephaly demonstrated some, but not all, the symptoms of this syndrome. Whether this boy showed a milder expression of the Weaver syndrome or benign familial macrocephaly is discussed.

Abnormalities, Multiple↗
PubMed1980

[Valvulography for the evaluation of the cerebrospinal shunt in childhood hydrocephalus (author's transl)].

Infection and obstruction of the cerebrospinal shunt are the most important disturbances in the management of childhood hydrocephalus. Often an occlusion of the shunt cannot be localized by conventional diagnostic means. Therefore the patency of Heyer-Systems was studied in 28 hydrocephalic children aged 2 months to 15 years with clinical signs of an increased intracranical pressure. The so-called valvulography with 99mTc-pertechnetate or Metrizamide (Amipaque). In more than 96% the results were confirmed during operation. The advantages of the X-ray method are: instrument and contrast medium are available at any time and the combination with cranial computertomography gives more precise data than scintigraphy alone.

Adolescent↗
PubMed1979

[Idiopathic osteolysis type Hajdu-Cheney in early childhood (author's transl)].

Severe clinical and radiological signs of the rare idiopathic osteolysis, type Hajdu-Cheney were manifest in a seven year old girl. A peculiar aspect of her face was evident since birth. The acroosteolysis and a general osteoporosis were radiologically visible when she was three years old. During the following 4 years there was a fast progression of the bone abnormalities and the shape of the skull became dolichocephalic.

Age Factors↗
PubMed1977

[The osteomyelitis of the hip in newborns and its functional treatment with "extensionsreposition" and "Hanausek-Retention" (author's transl)].

Since 1969 all our patients below the age of three years with congenital dislocation of the hip are treated by a modified overhead-traction (Extensionsreposition) and the "Hanausek-Apparat" for retention. Because of good results of careful reduction and retention in a mitigated Lorenz-position we also treated osteomyelitis of the hip-joint in newborns and following dislocation by this method. It is reported of 3 cases, different types of osteomyelitis of the hip and the method are described.

Bone Development↗
PubMed1976

Hypophosphatasia. Review of 24 cases.

Radiographic analysis of 24 cases of hypophosphatasia (H) from 9 Paediatric Centres was performed. 3 cases were of neonatal (lethal), 18 cases of infantile (severe) and 3 cases of late (benign) type. Some of the patients were in reality borderline cases between these groups. In the authors' material all the patients showed radiographic signs of the disease. These were divided into diagnostic, characteristic and suggestive features. All of the patients had in common generalised (usually irregular) osteoporosis, generalised (usually irregular) metaphyseal changes, craniostenosis (13 of 18 infantile cases) or widened cranial sutures and ofter bowing of the long bones. Besides the well know radiographic features of hypophosphatasia some less well known, rare or 'new' ones such as, 1. spurs of the long bones (Bowdler sign), 2. distal femoral central metaphyseal defects and epiphyseal defects, 3. S-like deformities of the tibiae, 4. abnormal shape of the distal phalanges of the fingers, 5. multiple rib fractures and slender bones, 6. wedging of the lower thoracic and upper lumbar vertebrae, 7. partial premature fusion of the epiphyses, 8. nephrocalcinosis, 9. loss of lamina dura around the teeth, 10. variation in radiographic appearances of a pair of siblings with lethal form, and, 11. rapid changes in roentgen appearances. are discussed. In two of our patients (siblings) phosphoethanolamine was undetectable in the urine. The authors doubt if a normal skeletal survey may be present at any stage in any of the three major types of hypophosphatasia.

Adolescent↗
PubMed1976

[Differential diagnostic problems resulting from massive doses of vitamin D and suspected pituitary dwarfism in the father of a child with vitamin-D-resistant rachitis (author's transl)].

A 1.8-year-old female child with retarded statomotor development and growth was reported. Radiologically and chemically, a rachitis was found which clearly improved following administration of 600,000 U. vitamin D3. Examination of the father who was thought to be suffering from pituitary dwarfism revealed hypophosphatemia and radiologic signs of osteomalacia. The diagnosis of a hypophosphatemic vitamin-D-resistant rachitis in this child could only be established with certainty during the course of the following months.

Adult↗