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Biomedical subjects

H J Manz

Publications and source records attributed to H J Manz.

At least 37 records · Page 2Linked to original sources
PubMed1989

Malassezia furfur--disseminated infection in premature infants.

Three infants, born prematurely, died after clinical illnesses of 67, 65, and 60 days from infection by Malassezia furfur. Each infant had been nourished with lipid emulsions delivered through deep-line catheters. The infections, all discovered at autopsy, were characterized by massive involvement of lungs. Two of the three had endocardial vegetations containing M. furfur; all three had lesions in liver, kidney, and spleen, and two had lesions in adrenal, pancreas, and colon. In addition, one of the infants had acute meningoencephalitis caused by M. furfur. In some of the distant organs, yeast cells of M. furfur were growing in the lumina of small vessels, filling the lumina, but causing no vasculitis or infarction. In addition to these benign collections of yeasts within vessels, there were acute inflammatory lesions as well. These were consolidation, vasculitis, granulomatous inflammation, septic thrombosis, and septic infarction of lung and foci of necrosis and inflammation in kidney and liver. Two previously reported autopsies described neonates with lesions in lung and heart. The authors' three cases for which autopsies were performed had lesions in lung and heart too but, in addition, had dissemination with acute lesions in kidney and liver. Finally, one patient had a severe meningoencephalitis caused by M. furfur.

Fat Emulsions, Intravenous↗
PubMed1989

MR imaging of neurocysticercosis.

Twenty-six patients with neurocysticercosis were studied with MR imaging to correlate their clinical presentation with the location and appearance of their neurocysticercosis lesions. Intraventricular cysts were present in 14 patients (54%), parenchymal cysts were present in 18 (69%), and intraventricular together with parenchymal cysts were present in six (23%). Intraventricular cysts were detected by mass effect, ventricular obstruction, detection of a cyst rim, and/or CSF flow void adjacent to the cyst. The intensity of most intraventricular and parenchymal cysts presumed to be viable was similar to that of CSF on both T1- and T2-weighted sequences. Cysts presumed to be degenerated had increased signal intensity on T1-weighted images, probably resulting from increased protein content. Pericystic high signal intensity surrounding lesions of various ages was seen on both proton-density- and T2-weighted images and represents gliosis, edema, and inflammation. Patients with parenchymal cysts had symptoms of seizures, while those with intraventricular cysts generally had symptoms related to obstructive hydrocephalus. Aqueductal stenosis, seen in 10 patients (38%), was possibly due to ependymal inflammation or adhesions caused by prior ventricular infection by neurocysticercosis. One patient with the racemose form of neurocysticercosis demonstrated abundant cyst wall proliferation resulting in obstructive hydrocephalus. In six patients scanned 1-6 months after oral praziquantel therapy, there was no change in the MR appearance of intraventricular cysts, while some parenchymal cysts showed evidence of degeneration. We found MR to be useful in detecting the cysts of neurocysticercosis and the accompanying signs of cyst degeneration and pericystic inflammation. MR was inferior to CT in the detection of parenchymal calcifications.

Adolescent↗
PubMed1989

Amniotic band syndrome. Report of two cases and review of the literature.

The amniotic band syndrome, a complex collection of asymmetric congenital anomalies, is almost certainly an underdiagnosed entity. No two cases are exactly alike. Two cases are reported, each of which exhibits craniofacial, visceral, body wall, and limb anomalies, the common types of defects seen in this syndrome. The literature is reviewed, and the most commonly accepted theory of pathogenesis is discussed in juxtaposition to another major theory. The importance of recognizing this syndrome is stressed, since it very rarely recurs in families.

Abnormalities, Multiple↗
PubMed1988

Cutaneous malignant melanoma in survivors of heritable retinoblastoma.

Six survivors of bilateral retinoblastoma developed cutaneous malignant melanoma 20 to 51 years (average, 31 years) after initial therapy for the ocular tumor. Five patients received radiation therapy to the orbital area. In two patients the cutaneous malignant melanoma developed in the field of irradiation. Two patients developed multiple cutaneous melanomas and are thought to have the dysplastic nevus syndrome. At this writing two patients are alive and well after wide resection of their skin tumors. A review of the literature suggests that cutaneous malignant melanoma accounts for about 7% of second malignant neoplasms in survivors of heritable retinoblastoma.

Adult↗
PubMed1988

Central spinal cord injury: magnetic resonance imaging confirmation and operative considerations.

A case of central cervical spinal cord injury, confirmed by magnetic resonance imaging (MRI) and treated by myelotomy, is presented. After recovering well from his central cord syndrome and walking with assistance, the patient developed a rapidly progressive myelopathy beginning 2 months after injury. His main injury localized clinically to the C8, T1 level; but central cord abnormalities were identified 3 months after injury at the C6 level by MRI: a high signal intensity on the proton density sequence and a low-signal intensity on the T1-weighted sequence. At operation 41/2 months after his injury and 1 month after complete paraplegia, a myelotomy at C6 failed to reveal any cavity (syrinx) but instead disclosed only intense gliosis inside a slightly atrophic spinal cord. Rapid clinical improvement ensued. Secondary syringomyelia may be an endstage condition after spinal cord insults that trigger a progressive, pathophysiological reaction leading to central cord necrosis. In selected cases, myelotomy may interrupt this MRI-identified, nosogenic process before cavitation has occurred.

Adult↗
PubMed1988

Intraparenchymal hemorrhage in preterm neonates: a broadening spectrum.

Among 800 neonates screened for cerebral bleeding, perinatal sonography identified 35 preterm neonates with intraparenchymal hemorrhage (IPH). The observed IPHs were categorized in five general groups: classical grade-IV hemorrhage (16 patients), grade-IV hemorrhage with coexisting but anatomically separate hemorrhages (four patients), grade-IV hemorrhage with later development of secondary parenchymal hemorrhages at distant sites (five patients), hemorrhagic periventricular leukomalacia (four patients), and parenchymal hemorrhage unrelated to grade-IV hemorrhages (six patients). Thirty IPHs had concurrent germinal matrix hemorrhages, but in only 16 patients did the IPH represent an extension of the subependymal hematoma and therefore qualify as pure grade-IV hemorrhage according to the most popular classification. The high concurrence of periventricular leukomalacia (80-100% in most groups) and germinal-matrix-remote IPHs supports the concept that most IPHs in preterm neonates represent secondary hemorrhages into ischemic brain tissue.

Brain↗
PubMed1987

An unusual case of central nervous system vasculitis.

A patient with CNS vasculitis limited to small vessels is described. Initial spinal cord involvement with paraparesis was followed by multifocal cerebral and cerebellar involvement, depicted by magnetic resonance imaging (MRI). She also had recurrent cerebral bleeding. A diagnosis of vasculitis was made by cerebral biopsy. Immunosuppressive therapy resulted in clinical improvement and resolution of the MRI abnormalities.

Biopsy↗
PubMed1987

Cytomegalovirus encephalitis associated with episodic neurologic deficits and OKT-8+ pleocytosis.

After 3 days of symptoms suggesting a viral illness, a 35-year-old man experienced three episodes of aphasia, right-sided sensory symptoms, and bifrontal headache. Each lasted several hours. CSF examination revealed a moderate lymphocytosis consisting of 80% OKT-8+ cells. Serum anti-cytomegalovirus (anti-CMV) antibody titer was elevated at 1:1,024 and subsequently fell to 1:64. Episodic symptoms recurred 5 months later, at which time the anti-CMV titer peaked at 1:8,192. A trial of inhaled oxygen aborted two episodes after several minutes each.

Adult↗
PubMed1987

Lymphocytic adenohypophysitis causing pituitary mass.

Lymphocytic infiltration of the anterior pituitary is a rare complication of pregnancy, which has been reported in 18 cases. Symptoms begin in the last trimester or soon after delivery. Radiologically, an expanding mass in the sella may result in suprasellar extension. We studied a case in which there was CT evidence of a pituitary mass 2 weeks postpartum, and biopsy confirmed the diagnosis.

Adult↗
PubMed1987

A review of the factors influencing the prognosis of medulloblastoma. The importance of cell differentiation.

The clinical, therapeutic, and histological features of 54 patients with medulloblastoma were analyzed retrospectively by a multivariate approach with regard to prognosis. The overall 5-year survival rate was 60%, with 48% of patients free of recurrence at 5 years. Cell differentiation, when present, was associated with a significantly longer recurrence-free period. Seventy-two percent of patients with the histological finding of cell differentiation were recurrence-free at 5 years. A marginally significant increase in the 5-year survival rate was also seen in association with differentiation. Only 34% of the patients whose tumor exhibited necrosis were alive at 5 years. There was no statistically significant difference in 5-year survival for children under 3 years of age or for the group of children aged 5 years or under. However, a significantly larger fraction (72%) of the group aged 5 years or under had a recurrence-free period of 5 years or more. Other factors including sex, extent of surgical resection. Chang tumor stage, posterior fossa radiation dose, and adjuvant chemotherapy did not influence prognosis.

Adolescent↗
PubMed1986

Primary degenerative dementia without Alzheimer pathology.

To define the pathology in cases of non-Alzheimer primary degenerative dementia (non-AD PDD), we have studied autopsies from four medical centres accessioned in consecutive years since 1976. Neurochemical studies of the basal forebrain-cortical (BF-C) cholinergic system have been conducted in cases from which frozen tissue was available. Twenty-two cases (mean age 70 years, range 47-86) in which the history was consistent with PDD, but which did not meet anatomic criteria for AD, were selected. Approximately 70 cases of PDD, which were accessioned in the same years and met the anatomic criteria for AD, were excluded. The pathologic findings permitted a classification into six groups: Lewy body disease (LBD), 4 cases; Pick's disease, 6 cases: cortical degeneration with motor neuron disease (CDmnd), 2 cases; hippocampal and temporal lobe sclerosis, 3 cases; few or nonspecific abnormalities, 5 cases; other disorders, 2 cases. Our findings suggest that LBD and Pick's disease account for a large proportion of cases of non-AD PDD in the presenile age group, but that a large number of other disorders occasionally present as PDD. Careful examination of the motor systems, as well as cerebral structures relate to cognitive function, is important in the neuropathologic evaluation. Lesions of the BF-C cholinergic system have been most consistent and severe in LBD, and have not been identified in CDmnd.

Aged↗
PubMed1986

Cortical degeneration with swollen chromatolytic neurons: its relationship to Pick's disease.

We report two cases of dementia in which cortical degeneration with widespread swollen chromatolytic neurons (SCN) was the dominant pathologic feature. Each patient had received the diagnosis of Alzheimer's disease on the basis of clinical findings. There was no deficit of cortical choline acetyltransferase activity, assayed in one case, or lesions of the nucleus basalis of Meynert. The brains had moderate to marked frontal atrophy. Comparison of SCN with several other cerebral degenerative disorders indicates a similarity with certain features of the transmissible spongiform encephalopathies and with corticodentatonigral degeneration. The pathologic features of our cases are those of a number of other cases reported as "Pick's disease," and may represent an earlier stage in the pathogenetic process than the severe, sharply circumscribed atrophy with "nonspecific" cell loss and gliosis as the only microscopic residuals. Our findings re-emphasize the need to search for pathogenetically distinct subgroups which have been wholly or partially subsumed into the concept of Pick's disease.

Acetylcholine↗
PubMed1986

Trigeminal herpes zoster causing mandibular osteonecrosis and spontaneous tooth exfoliation.

We have described a 60-year-old woman with polymyalgia rheumatica treated initially with prednisone but ultimately with azathioprine and cyclophosphamide. Herpes zoster infection along the mandibular division of the right trigeminal nerve ensued with spontaneous exfoliation of two teeth. She subsequently died. Medical practitioners need to be made aware of this rare complication of a common disease.

Azathioprine↗
PubMed1986

Hepatocellular carcinoma in an 11-year-old girl with Fanconi's anemia. Report of a case and review of the literature.

An 11-year-old girl with Fanconi's anemia, who died of Corynebacterium septicemia, was found at autopsy to have a solitary, previously undiagnosed hepatocellular carcinoma (HCC). Although the association between Fanconi's anemia and malignancies such as leukemia and squamous cell carcinoma is well documented, its relationship to HCC remains controversial and obscure. Anabolic steroid therapy for Fanconi's anemia has also been considered a promoter for hepatocellular neoplasms. This report documents the youngest known patient with Fanconi's anemia to develop HCC and discusses the association between these conditions.

Anemia, Aplastic↗
PubMed1986

Phenytoin-induced systemic granulomatous vasculitis.

Much has been written about adverse effects accompanying the use of phenytoin. However, the rare occurrence of systemic vasculitis induced by this drug has only been the subject of sporadic case reports and has not truly been characterized as a definitive entity. The high fatality rate of this complication is a proof of its severity. A case of multiorgan necrotizing granulomatous vasculitis related to the use of phenytoin is reported and the clinicopathologic aspects of seven similar cases assembled from the literature are summarized and discussed. The most commonly affected patients are elderly black men who present with skin rash, fever, and, frequently, eosinophilia. Tissue examination discloses systemic vasculitis that is often granulomatous and involves mainly the liver, kidneys, and spleen. Immediate recognition of this diagnosis is stressed, since proper treatment may reverse a fatal clinical course.

Adult↗