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Biomedical subjects

H Mena

Publications and source records attributed to H Mena.

At least 37 records · Page 2Linked to original sources
PubMed1994

Pneumatosis cystoides intestinalis in systemic lupus erythematosus with intestinal vasculitis: treatment with high dose prednisone.

Pneumatosis cystoides intestinalis (PCI) is an uncommon disorder usually associated with intestinal and pulmonary obstructive diseases, recent abdominal procedures and systemic illnesses. PCI has been reported in patients with systemic lupus erythematosus associated with intestinal vasculitis. We describe herein a patient with a month history of intermittent abdominal pain, diarrhoea, hyporexia, and weight loss who underwent intestinal resection for acute abdomen. Post-operatively she gave a three-month history of arthritis of the right knee, ankles and feet, arthralgia of the wrists, MCPs and shoulders. She also described weakness, weight loss, Raynaud's phenomenon, and a skin rash. Laboratory examination revealed an increased ESR, low haemoglobin and haematocrit, positive rheumatoid factor, a positive ANA with a speckled pattern, as well antibodies to DNA, SS-A and cardiolipin. The abdominal symptomatology especially pain, cramps and bouts of diarrhoea persisted after the surgery and became worse two months later. Abdominal X-ray showed distention of bowel with cyst formation in the wall of the entire colon. A diagnosis of PCI was made radiologically. The intestinal pathology was reviewed and vasculitis was identified. The patient received treatment with high dose prednisone with an excellent response; prednisone was progressively tapered and she has been asymptomatic without abdominal complaints or other symptoms for over a year.

Adult↗
PubMed1993

A 5' splice junction mutation leading to exon deletion in an Ashkenazic Jewish family with phosphofructokinase deficiency (Tarui disease).

A deficiency of the muscle isoform of the enzyme, phosphofructokinase (PFK, EC 2.7.1.11), leads to an illness (glycogenosis, Type VII) characterized by myopathy and hemolysis. A patient with this disease and an affected sister were found to have a G to A substitution at the 5' donor site of intron 5 of the PFK-M gene. This mutation led to a splicing defect: a complete deletion of the preceding exon in the patient's mRNA. The patient, an affected sister, and related and unrelated family members, who were of Ashkenazic Jewish background, were screened for the mutation by denaturing gradient gel electrophoresis and by allele specific hybridization of genomic DNA. The affected sisters are homozygous for the mutation, and their children, who are unaffected, are heterozygous. The only previously characterized genetic defect in this disease, found in a Japanese patient, was a G to T mutation at the beginning of intron 15 with splicing to a cryptic site within exon 15 (1). Both mutations lead to inframe deletions, but of different parts of the protein. The differences between the two aberrant proteins may account for clinical differences between our patients and the Japanese patient.

Base Sequence↗
PubMed1992

Pineal region masses: differential diagnosis.

Most pineal region masses are malignant germ cell neoplasms that occur in young male patients. The most common is a germinoma, which is a homogeneous mass with signal intensity and attenuation similar to those of gray matter; the mass engulfs a densely calcified pineal gland. Teratomas are multilocular heterogeneous masses containing lipid areas. Other types of pineal region masses include choriocarcinoma, endodermal sinus tumor, and embryonal carcinoma. Pineal parenchymal neoplasms are usually either pineocytomas or pineoblastomas, which may "explode" preexisting pineal calcifications. Unlike the germinomas, they have no sexual predilection and may be seen in patients who are 20 years of age or older. Although the correct histologic diagnosis may be suggested with a careful evaluation of the morphologic features, attenuation, and signal intensity characteristics, very few of these tumors have a truly pathognomonic imaging pattern. Thus, histologic verification is necessary for most pineal region masses that appear to be neoplastic.

Brain Diseases↗
PubMed1991

Hemangiopericytoma of the central nervous system: a review of 94 cases.

Ninety-four cases of central nervous system hemangiopericytoma (CNS-HPC) are reported. Hemangiopericytoma was found more commonly in men than in women. The mean age at diagnosis was 40.9 years for men and 47 years for women. The tumor was found throughout the entire CNS, usually superficially and closely related to the meninges. Based on multiple histologic variables, the original tumors were divided into differentiated (n = 67) and anaplastic (n = 27). Anaplastic HPC was characterized by the presence of necrosis and/or greater than five mitoses per ten 400x microscopic fields, and at least two of the following microscopic features: hemorrhage, moderate to high nuclear atypia, and moderate to high cellularity. For those patients known to be dead, median survival time was 144 months for differentiated HPC and 62 months for anaplastic HPC. Fifty-seven (60.6%) patients had one or more recurrences and metastasis developed in 22 (23.4%). Thirty-five of 56 patients with differentiated HPC had recurrence, while 22 of 26 patients with anaplastic HPC had recurrence. Bone, liver, lung, central nervous system, and abdominal cavity were the most common sites of metastasis. Postoperative radiotherapy and/or chemotherapy were significantly associated with increased patient survival time.

Adolescent↗
PubMed1991

Primary angiosarcoma of the central nervous system. Study of eight cases and review of the literature.

Angiosarcoma arising in the central or peripheral nervous system has rarely been reported. Eight patients with primary angiosarcoma of the central nervous system are described here; these included five males and three females ranging in age from 2 weeks to 72 years (mean 38 years). Of the eight neoplasms, six were located in the cerebral hemispheres and one was in the meninges; the site was unknown in the other. All patients underwent surgical resection. Five of the eight patients died, four within 4 months after surgery and one after 30 months. Two of the remaining three patients were 17 and 27 years old at the time of diagnosis and were alive at follow-up review 39 and 102 months after surgery, respectively. One patient was lost to follow-up monitoring. Microscopically, all eight tumors demonstrated a well-differentiated pattern with irregular vascular channels and intraluminal papillae; in addition, four showed poorly differentiated solid areas. Immunohistochemical staining of neoplastic cells to factor VIII-related antigen and Ulex europaeus agglutinin I was performed in five tumors and was focally positive in four. No correlation could be shown between the histological features and the growth and biological behavior of the tumors.

Adolescent↗
PubMed1989

A histologic and immunocytochemical study of choroid plexus tumors of the dog.

Sixteen choroid plexus (CP) tumors in 12 male and four female adult dogs were analyzed microscopically. Tumors were in the lateral (six), third (six), and fourth (four) ventricles. The average age of the dogs was 6 years. Tumors were classified by the following criteria: 1) choroid plexus papilloma (CPP), which resembled normal choroid plexus and had low mitotic activity; 2) choroid plexus papilloma (CPP), which resembled normal choroid plexus and had low mitotic activity; 2) choroid plexus papilloma with atypical features (atypical CPP), which had increased cellular density, nuclear atypia, two to four mitoses per 40x microscopic field, necrosis, and infiltration of the brain parenchyma and/or leptomeninges; and 3) choroid plexus carcinoma (CPC), which had marked nuclear atypia, poorly formed papillae, greater than four mitoses per 40x microscopic field, abnormal mitotic figures, and/or extraneural metastasis. The 16 tumors were classified either as CPP or atypical CPP (none as CPC). Statistically significant associations between brain infiltration and necrosis and atypical CPP were identified. Immunohistochemical studies in 11 tumors demonstrated staining for keratin in three tumors, two of which also reacted with carcinoembryonic antigen (CEA). There was no immunoreactivity with glial fibrillary acidic protein or epithelial membrane antigen. Choroid plexus from one of three control dogs stained focally for cytokeratin only. It is concluded that normal choroid plexus and CP tumors in the dog express epithelial, but not glial differentiation.

Animals↗
PubMed1985

Giant fibrosarcoma of the ovary.

An unusual case of a huge primary ovarian fibrosarcoma, the largest tumor of its type reported to date, is presented. The gross, light microscopic, and ultrastructural features are described. The tumor was unique in three aspects: (a) its massive bulk; (b) the prompt relief of symptoms after debulking; and (c) most significantly, the lack of extensive abdominal metastasis in spite of its high grade, large size, and 6-month delay in resection.

Female↗
PubMed1984

Transverse myelitis from intraarterial penicillin.

A child was given intramuscular benzathine penicillin and experienced manifestations of sudden, irreversible transection of the spinal cord in the lower thoracic region. The biopsy supported an intravascular injection with occlusion of the spinal vasculature as the etiological mechanism. A review of similar cases reveals a recurring pattern--intramuscular injection with standard techniques and sites into a small muscle mass without evident blood return followed by rapid progression of paralysis. The problem seems to turn upon an inability to recognize the inadvertent intraarterial injection.

Humans↗
PubMed1984

Gallium-67 citrate localization in the heart secondary to constrictive pericarditis with myocardial fibrosis.

Scintigraphy has demonstrated gallium-67 accumulation within the heart in pericarditis and cardiomyopathies of various kinds. We present a case report of a 63-yr-old man with multisystem disease who showed extensive myocardial uptake of Ga-67 by scintigraphy. At autopsy, constrictive pericarditis with myocardial fibrosis was found. Gallium-67 localization has not been documented previously in myocardial fibrosis accompanying constrictive pericarditis.

Cardiomyopathies↗
PubMed1983

A sibship with neuroaxonal dystrophy and renal tubular acidosis: a new syndrome?

The clinical and pathological features of three siblings from nonconsanguineous parents are described. Two of the children died at 6 1/2 and 7 months of age of a central nervous system disorder characterized by severe behavioral retardation, hypotonia, and pyramidal tract signs. The third child, now 8 years old, has global mental retardation, inability to talk, marked tremors, and gait disturbances. All children exhibited inability to concentrate or acidify the urine, with growth delay partially corrected in the living child by alkali therapy. Computed tomographic scans revealed hyperdense lesions in the thalamus of one of the siblings and more striking hyperdensity of the basal ganglia in the eldest child. Findings at postmortem examination in two children, and by renal and sural nerve biopsy in the third, include swollen axons in the internal capsule and peripheral nerves, and neurons with iron-staining deposits, gliosis, and macrophages containing lipofuscin pigments in the brain. The renal findings include material positive for periodic acid-Schiff and hyperdense granular deposits in renal tubules.

Acidosis, Renal Tubular↗
PubMed1981

Central and peripheral myelinopathy associated with systemic neoplasia and chemotherapy.

Three adult patients with leukemia and a patient with cutaneous melanoma were treated with a variety of therapeutic agents administered systemically. Three of these patients received either cytosine arabinoside or thio-TEPA, by intrathecal injection and radiotherapy to the cranium or spine. Three patients developed progressive motor and sensory deficits and the fourth became confused and disoriented. These symptoms were chronologically related to the time when chemotherapy was begun. Death occurred 11/2, 2, 5 and 7 months, respectively, after the beginning of neurologic deficit. The spinal white matter showed vacuolation, myelin disintegration, axonal swelling, fibrillary gliosis, and infiltration by macrophages. Vesicular disintegration of the myelin lamellae seems to be the earliest lesion affecting both central and peripheral myelin. Gliosis and macrophages were visible only in the two patients who survived at least five months from the time of the neurologic deficit.

Adult↗
PubMed1978

Diffuse microglial proliferation after global ischemia in a patient with aplastic bone marrow.

Abundant proliferation of cells having the histologic and tinctorial features of microglia, were seen in the brain of 34-year old man who suffered cardiac arrest 10 days before death and whose bone marrow was nearly totally depleted of white-blood-cell precursors. It is suggested, that in adult human brains there are native microglial precursors which can take on a phagocytic function. Therefore, brain macrophages do not have to originate from circulating monocytes, as suggested by some animal studies.

Adult↗
PubMed1978

Primary brain sarcomas: light and electron microscopic features.

The following is a description of the diagnostic structural features in three neoplasms in which we diagnosed: primary intraparenchymal brain sarcoma. One appeared to have arisen de novo from the mesenchymal components of the brain parenchyma and was labeled fibrosarcoma. A second case, having mixed features of meningioma and fibrosarcoma, may represent an instance of sarcomatous changes in a prexisting intraparenchymal meningioma. The third sarcoma's ultrastructural characteristics were those of embryonal capillaries and a designation of hemangiosarcoma is proposed for that one. An angiographic, preoperative diagnosis of meningioma was suggested in all three instances. Two of the patients died a few weeks after craniotomy and the third one is alive and well eighteen months after resection of the neoplasm.

Adolescent↗