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Biomedical subjects

H Ohnacker

Publications and source records attributed to H Ohnacker.

At least 19 recordsLinked to original sources

Lung hypoplasia and severe pulmonary hypertension in an infant with double heterozygosity for spondyloepiphyseal dysplasia congenita and achondroplasia.

A rare instance of double heterozygosity for spondyloepiphyseal dysplasia congenita and achondroplasia is presented. Despite midface hypoplasia, thorax deformity and lung hypoplasia, the child survived the neonatal period. Severe pulmonary hypertension, already present at birth, led to right heart failure and death at the age of 1 year.

Achondroplasia

[Leiomyosarcoma of the breast 16 years following successful treatment of a rhabdomyosarcoma of the orbit in childhood].

A case of a mammary leiomyosarcoma in a 23-year-old woman is presented. The tumor appeared 16 years after successful treatment of an embryonal rhabdomyosarcoma of the orbit. Rhabdomyosarcomas are the most frequent soft tissue tumors of childhood, the orbit and the paratesticular region being the most common primary site for this tumor. In contrast, leiomyosarcomas other than those evolving from the viscera or the urogenital organs are rare neoplasms at any age. With the improvement of cancer treatment and survival rates, the risk of late effects after successful treatment for malignant tumors during childhood is increasing. Growth, development and fertility may be impaired and cosmetically disturbing facial and dental complications are common. Development of novel primary tumors is a known further consequence of successful treatment of brain tumors, retinoblastoma and acute leukemias. This is the case when high dose local radiation therapy and/or chemotherapy, especially alkylating agents, were used. Development of novel primary tumors is also known after treatment of childhood rhabdomyosarcomas. This report is intended to show that a second primary tumor may occur many years after a first successfully treated malignant neoplasm, and that young people are at risk for development of tumors at sites that are uncommon to this age group.

Adult

[Plasma cell granuloma of the lung].

Isolated primary tumors of the lung are rarely seen in childhood and are benign in one third of the patients. Plasma cell granulomas represent the most common benign tumor of the lung in this age group. Histologically, the tumor is characterized by the various components of a chronic inflammation or granulation tissue. Frozen sections can pose difficulties in the differentiation between a malignant and benign process. In view of the case history of our patient we set out to demonstrate that a knowledge of the clinical picture and a correct interpretation of associated laboratory findings facilitate diagnosis. To our knowledge we describe for the first time the ultrastructural findings of so-called "microtubule-reticular structures" giving further evidence of the inflammatory origin of this lesion.

Child

[Cervical thymic cyst in childhood].

In three children (2, 4 and 10 years of age) a cervical thymic cyst was removed. We found multilocular cysts with xanthochromic luminal fluid covered with a fibrous capsule. Histological features are lymphatic follicles, partly atrophic, as well as regular thymic tissue with cortex, marrow and Hassall's corpuscles.

Child

Mesenterial histiocytic necrotizing lymphadenitis. Case report.

A 14-year-old girl was hospitalized with fever, jaundice, vomiting and right sided abdominal pain. A laparotomy was performed because of muscular defence and ascites. There was a mass of enlarged red and blue colored lymph nodes in the mesentery of the lower ileum loop. The histologic diagnosis of HNL without granulocytic infiltration was made. A septic-toxic shock developed after surgery. Respiratory insufficiency necessitated the use of a respirator, and acute renal failure with oliguria made hemodialysis necessary. The dramatic clinical course of the illness and the localization of the affected lymph nodes in the abdomen are unusual for an HNL; the lack of granulocytic infiltration contradicts the clinical picture of a bacterial infection. Neither a bacterial nor a viral pathogen could be found. However, the patient had been treated with antibiotics before.

Adolescent

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

[Ganglion in the discus triangularis. First description].

A fifty-four year old patient had persisting pain in the wrist following a distal radius fracture two years previously. A perforation of the discus triangularis was confirmed by arthrography, and the disc removed. Gelatinous fluid was found in the joint at operation. Histologically, a ganglion was present in the disc, close to an old tear. As far as we know, this is the first report of a disc ganglion. Disc ganglia probably occur more frequently than is recognized and could be responsible for pain, swelling and recurrent wrist ganglia.

Cartilage, Articular

[How reliable is cancer diagnosis with needle biopsy of the prostate?].

Aim of the study was to investigate the reproducibility and the reliability of the morphological diagnosis, of different morphological parameters and representativity of needle biopsy material compared with transurethral resection material. 416 needle biopsies of carcinomas of the prostate were studied by 5 pathologists. 130 biopsies were evaluated twice by the same investigators and 325 at least by one other investigator. In 65 cases, tissue obtained by transurethral resection was evaluated by the same investigator who had seen the biopsy. The diagnosis of carcinoma of the prostate was highly reproducible, only in 2% of the cases the original diagnosis was not confirmed; 5 out of 27 systematically evaluated parameters proved to be very reliable and reproducible, i.e. histological tumor classification, cribriform glands, invasion of perineural spaces, nucleoles, nuclear polymorphism, 8 parameters were sufficiently reproducible and 14 were unreliable among which cytologic differentiation was one. The diagnostic reproducibility between biopsy material and transurethral resection material was only 48.8%. This very low figure can be explained by the marked histologic heterogeneity of the tumors of which only 13% were classified as uniform carcinomas. The conclusions of the investigation are: 1. The diagnosis carcinoma is highly reliable and reproducible in needle biopsies. 2. Needle biopsy tissue is not representative for the entire tumor. 3. Grading of prostate carcinoma is highly subjective and therefore no sound basis for therapy and prognosis in the individual patient.

Biopsy, Needle

[Diagnostic accuracy of prostate needle biopsies in cancer of the prostate].

The carcinoma of the prostate can be diagnosed with high certainty in needle biopsy. Among 450 cases only one false diagnosis was found. Under routine diagnostic conditions, semiquantitative evaluation showed that 50% of the investigated morphological parameters in carcinomas of the prostate can be reproduced by one and the same or different investigators with a sufficiently high degree of certainty. Comparison between needle biopsy and transurethral resection material showed that the overall coincidence of tumor classification (according to the predominant part of tumor) was only 50%. The authors conclude that tumor therapy should not predominantly be based on morphology present in a single needle biopsy.

Biopsy, Needle

[Prognostic significance of clinical and morphological parameters in prostate cancer].

In 269 patients with carcinoma of the prostate, a retrospective statistical evaluation was done to correlate prognosis with different clinical and morphological criteria. All prostate biopsies were reviewed, the clinical staging redone and compared to survival of the patients. The most reliable criteria for prognostic prediction are clinical staging (rectal palpation, radiological examination and prostatic acid phosphatase). The morphologic parameters (histologic type of tumor, perineural invasion and perifocal inflammation) are of additional but of limited value.

Age Factors