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Biomedical subjects

F C Chu

Publications and source records attributed to F C Chu.

At least 37 records · Page 2Linked to original sources

Acquired nystagmus in early childhood: a presenting sign of intracranial tumor.

This study is a multicenter, retrospective report of 10 infants in whom acquired nystagmus was the initial sign of chiasmal/ parachiasmal glioma. Nine patients presented before the age of 10 months. The nystagmus, primarily described as pendular and asymmetric, was difficult to differentiate from and therefore most often diagnosed as spasmus nutans . On average in the ten patients, the intracranial glioma was not recognized for 8.6 months after the onset of nystagmus. In the five diagnosed as spasmus nutans , the mean delay in recognizing the tumor was 14.5 months. Three associated clinical findings were present or developed in these patients to distinguish this entity from spasmus nutans : optic atrophy in all ten patients, poor feeding due to diencephalic syndrome in 5 of 10, and increased intracranial pressure with hydrocephalus in 3 of 10. The acquired nystagmus in these infants was evidence of a life-threatening chiasmal/ parachiasmal glioma.

Astrocytoma↗

The pathology of pingueculae in Gaucher's disease.

Several reports have noted an apparent association between pingueculae and Gaucher's disease and suggested that the yellow coloring of these lesions is attributable to Gaucher cells. The authors biopsied pingueculae from ten patients with this disorder and examined them by serial light microscopy and electron microscopy. They failed to find Gaucher cells in any specimen, but, as typical for pingueculae, the elastosis observed histologically could account for their yellow hue.

Conjunctiva↗

Leishmaniasis affecting the eyelids.

Leishman-Donovan bodies were recognized in the smear of a biopsy specimen from an eyelid ulcer. The infecting organisms were identified serologically as Leishmania braziliensis panamensis. The ulcer responded to pentavalent antimony. Ultrastructurally, the organisms had double-unit membranes, beneath which lay a palisade of microtubules. At one end of the organism, there was a rudimentary flagellum; at the other, the nucleus. A kinetoplast basal complex separated the two.

Adult↗

Clinical studies of color vision with Gunkel's chromagraph.

Color thresholds in a series of patients with local or systemic diseases were determined by a chromagraph method and subjected to computer analysis. When compared with normal persons, those with optic nerve disease (multiple sclerosis, optic neuritis, and optic atrophy) showed an overall weakness for all colors (enlarged neutral areas), with an additional specific defect in the orange-cyan (greenish blue) axis. Those with the two retinal diseases studied (macular degeneration and retinitis pigmentosa) also showed threshold elevation for all colors, but with a special defect in the yellow-blue axis. The general elevation was greater for patients with retinitis pigmentosa than for those with macular degeneration, regardless of the visual acuity. In patients undergoing treatment for systemic lupus erythematosus and rheumatoid arthritis, there was a mild elevation of the color threshold, especially for yellow.

Arthritis, Rheumatoid↗

Macula halo syndrome. Variant of Niemann-Pick disease.

The macula halo syndrome is the name proposed to describe patients with a unique ring-form opacity about the foveolas and a histiocytic storage disease. Since sphingomyelinase deficiency has now been found in the three patients in whom it was sought (including two in the present report), the entity may be classified as a variant of Niemann-Pick disease. A secondary hyperlipidemia may also be present. The macula halos consisting of symmetric crystalloid opacities with little or no visual impairment are pathognomonic of the entity.

Adult↗

Radiation induced sarcomas of bone following therapeutic radiation.

Because of new therapeutic trends of multi-modality and the importance of late effects, we have updated our series of radiation induced bone sarcomas seen at Memorial Sloan-Kettering Cancer Center over the past four decades. A total of 37 cases of bone sarcoma arising from normal bone in the irradiated field was analyzed. The median for latent period from irradiation to diagnosis of bone sarcoma was 11 years with a minimum latent period of four years. The median radiation dose for the bone sarcoma was 6000 rad in 6 weeks with a minimum total radiation dose of 3000 rad in 3 weeks. We have found nine patients who developed bone sarcomas in the radiation field after successful treatment of Hodgkin's disease. Criteria for radiation induced bone sarcomas and the magnitude of the risk of bone sarcomas are briefly discussed.

Bone Neoplasms↗

Hyperfractionated total body irradiation for bone marrow transplantation. Results in seventy leukemia patients with allogeneic transplants.

From May, 1979 to March, 1981, 76 leukemia patients were prepared for bone marrow transplantation (BMT) with a new hyperfractionated total body irradiation (TBI) regimen (1320 cGy in 11 fractions, 3x/day), followed by cyclophosphamide, 60 mg/kg, for two days. Partial lung shielding was done on each treatment, with supplemental electron beam treatments of the chest wall to compensate, and of the testes, a sanctuary site. This regimen was initiated to potentially reduce fatal interstitial pneumonitis as well as decrease leukemic relapse. These patients were analyzed in May, 1982, for a minimum follow-up of 14 months. Overall actuarial survival at 1 year for acute non-lymphocytic leukemia (ANLL) patients is 63%, while relapse-free survival at 1 year is 53%. For those ANLL patients who underwent BMT while in remission (first, second, and third combined), relapse-free survival is 61% at 1 year compared with 40% for those patients who had their BMT at the time of relapse (greater than or equal to 10% blasts in marrow). On the other hand, for acute lymphocytic leukemia (ALL) patients, there is no significant difference between relapse or remission patients with regard to overall survival or relapse-free survival, when relapse is defined as greater than 5% blasts in the marrow at the time of cytoreduction. Overall actuarial survival at 1 year for ALL is 61% and relapse-free survival is 45% at 1 year. Patients with ALL who had their BMT cytoreduction at the time of relapse have a survival equal to that of our remission patients, and greater than that of patients in relapse cytoreduced with a single dose as reported by others. However, patients with greater than or equal to 10% blasts have not fared as well, having only a 22% 1 year relapse-free survival compared with a 68% 1 year relapse-free survival for patients with less than 10% blasts. Fatal interstitial pneumonitis has dropped to 18% compared with 50% in our previous single-dose TBI regimen (1000 cGy), in which the same doses of cyclophosphamide were given prior to TBI. In conclusion, not only has fatal interstitial pneumonitis been reduced by hyperfractionation and partial lung blocking, but there may be a survival advantage in ALL patients in relapse, who have a survival equal to that of remission patients. This may indicate a greater cell kill with the higher dose (1320 cGy) attained with this regimen, in these patients with a higher leukemic cell burden.

Adolescent↗

The familial occurrence of cutaneous melanoma, intraocular melanoma, and the dysplastic nevus syndrome.

The occurrence of cutaneous melanoma and intraocular melanoma as double primary cancers in the same patient and in different members of the same family has suggested that these two forms of melanoma are etiologically related. It is theoretically possible that the link between these two pigment cell malignancies may be the dysplastic nevus syndrome, and patients with the dysplastic nevus syndrome may have an increased risk of intraocular melanoma and cutaneous melanoma. We studied two new kindreds in which cutaneous melanoma, intraocular melanoma, and the dysplastic nevus syndrome occurred and conducted neuro-ophthalmologic examinations of 26 patients with hereditary cutaneous melanoma or the dysplastic nevus syndrome, or both. In the one family studied in detail, the cutaneous melanoma predisposition came from the paternal bloodline, whereas the intraocular melanoma occurred in the maternal bloodline. The ophthalmologic examinations disclosed neither intraocular melanoma nor suspicious or atypical choroidal nevi. Our limited data suggested that the association of intraocular melanoma with cutaneous melanoma and dysplastic nevus syndrome may be coincidental.

Adult↗

The pathology of idiopathic midline destructive disease (IMDD) in the eyelid.

Subcutaneous eyelid and brow nodules were noted in a 25-year-old man who, at the age of 14 years, had developed the central facial and upper airway necrosis characteristic of idiopathic midline destructive disease (IMDD). One large nodule was located near the lateral canthus rather than a midline position. Insofar as we know, this report would constitute the first evidence of IMDD in the eyelids and brow in the absence of orbital disease.

Adult↗

Blink-saccade synkinesis.

We studied two patients who could make saccades of normal velocity and amplitude only in association with a simultaneous blink. In one patient, the initiation of saccades was also facilitated by blinks. Both patients had signs of cerebellar or brainstem dysfunction, suggesting a posterior fossa localization for blink facilitation of saccadic velocity and amplitude.

Adult↗

Testicular cancer in cryptorchids.

One-hundred thirty-seven patients with a history or clinical evidence of cryptorchidism and testicular germinal tumor were treated at our hospital from 1934 to 1976. Cryptorchidism was corrected ipsilaterally or contralaterally in 93 patients with intrascrotal testis cancer when they were from 2 to 42 years old, either spontaneously (24 patients), by orchiopexy (58 patients), or by hormonal therapy (11 patients). Forty-four cryptorchid patients (uncorrected cases) had either ipsilateral inguinal (24 patients), or abdominal (14 patients), or contralateral intrascrotal tumors (six patients). Tumor histologic types on orchiectomy were pure seminoma in 56 patients, embryonal carcinoma in 41, teratocarcinoma in 37, and pure choriocarcinoma in 3. The five-year survival rates were similar in the corrected (61%) and uncorrected (63%) cases, and they were higher in patients with pure seminoma (79%) than in patients with germinal carcinomas (50%). The majority (64 of 80) of five-year survivors received regional lymphatic irradiation in 41 patients with pure seminoma and/or systemic chemotherapy in 23 patients with other germinomas. Since the testicular tumors that developed despite correction of the cryptorchid state were predominantly (72%) germinal carcinomas, unilateral cryptorchidism, which usually is associated with testicular atrophy, should be treated by orchiectomy instead of orchiopexy to prevent ipsilateral carcinogenesis. Cryptorchid patients with testicles that descended late should be observed periodically, especially after the 20-year latent period, for early detection of cancer.

Adolescent↗

Ocular signs of cerebellar disease.

Ocular signs of cerebellar disease have been increasingly appreciated with the advent of means for quantitative recording of eye movements. The graphs in this article illustrate ocular flutter, dysmetria, abnormal (nonsmooth) pursuit, instability of fixation, faulty vestibular suppression, impaired optokinetic response, end-position nystagmus, and rebound nystagmus. The signs may be categorized as follows: (1) proprioceptive abnormalities manifest by flutter, dysmetria, and instability of gaze and (2) defects of vision-dependent functions manifest by abnormalities of pursuit, vestibular suppression, optokinetic response, and nystagmus.

Basal Ganglia↗

Therapy and prognosis of testicular carcinomas in relation to TNM classification.

Eight-hundred and thirty-one patients with testicular carcinomas, either teratocarcinoma (405), embryonal carcinoma (406) or pure choriocarcinoma (20), treated mainly at our center from 1950 to 1976, were clinicopathologically staged according to the TNM Classification. The cancer was confined to the body of testis alone (T1 N0 M0) or extended to paratesticular structures (T2-4 N0 M0) in 37% of all patients. Para-aortic lymph nodes were found involved (N1-3) in 33% and juxtaregional lymph nodes (N4) in 9% of patients; distant metastases were detected initially in the lung alone (M1) and other distant organs (M2) in 21% of the patients. Postorchiectomy treatment was retroperitoneal lymphadenectomy with or without regional-juxtaregional irradiation and systemic chemotherapy in 470 patients; the other 361 patients received external irradiation and/or adjuvant chemotherapy. Survival determined at 5 years was 58% in teratocarcinoma cases, 41% in embryonal carcinoma cases and 0% in pure choriocarcinoma cases. Rates of 5-year survival according to the TNM staging were 81% for T1 N0 M0 tumors, 58% for T2-4 N0 M0 tumors, 44% for N1-3 M0 tumors, 33% for N4 M0 tumors and 10% for N0-4 M1 or 2 tumors. In patients who underwent lymphadenectomy with or without external irradiation, the 5-year survival rates with and without adjuvant chemotherapy, respectively, were 96% and 86% for T1 N0 M0 tumors, 100% and 60% for T2-4 N0 M0 tumors, 66% and 42% for N1-3 M0 tumors, 54% and 40% for N4 M0 tumors and 38% and 0% for N0-4 M1 tumors. In patients treated by external irradiation alone or following lymphadenectomy the rates of 5-year survival with versus without adjuvant chemotherapy were 100% versus 66% for T1-4 N0 M0 tumors, 44% versus 18% for N1-3 M0 tumors, 41% versus 22% for N4 M0 tumors and 3% versus 4% for N0-4 M1-2 tumors.

Adolescent↗

Total skin electron beam therapy for cutaneous lymphomas and leukemias.

Total skin electron beam therapy (TSEB) was used in the treatment of 33 patients with lymphoma and 13 patients with leukemia involving extensive segments of the skin surface. Twenty-two of 23 had skin lesions as a primary manifestation of lymphoma (primary cutaneous lymphoma--PCL) and 11 developed cutaneous lesions following disseminated nodal lymphoma (secondary cutaneous lymphoma--SCL). A once weekly fractionation scheme was employed to irradiate the entire skin surface with 3.5 to 4 MeV electron beam from a 6 MeV linear accelerator. During each weekly session, 400 rad were delivered to the entire skin and a complete course consisted of 4-6 consecutive weekly sessions. The majority of patients have been previously treated elsewhere for various periods and all patients have been at risk for a median of 12 months, range from 12-117 months following TSEB. Striking predominance of the diffuse pattern (76%) was demonstrated in both the PCL and SCL. There was extracutaneous involvement in 63% (13/22) of the PCL, nodal or visceral at onset of TSEB; median follow-up was 24 months, range 6-117 months; 20/22 (90%) of all patients obtained prompt relief of symptoms and complete regression of cutaneous lesions. Duration of cutaneous remission ranged from 6-96 months, median 18 months; in general, duration was adversely influenced by the presence of visceral involvement at onset of TSEB. Although cutaneous response among the patients with SCL and leukemia was equally good, many of these patients were treated for palliation because of rapid progression of their disease. Once weekly treatments were highly effective, well-tolerated and no untoward immediate or late effects have been noted in the bone marrow or normal skin irradiated.

Adolescent↗

Radiation therapy before cystectomy in the management of patients with bladder cancer.

During the preceding two decades, 309 patients with bladder cancer were treated by irradiation before cystectomy at the Memorial Sloan-Kettering Cancer Center. A radical tumour dose averaging 6000 cGy (rad) in six weeks was given to 104 patients who underwent radical cystectomy +/- one year later for persistent or recurrent cancer. Pre-operative pelvic irradiation was planned in 205 patients who received either 4000 cGy in four weeks (119) or 2000 cGy in one week (86) and underwent radical cystectomy after +/- six weeks and two days, respectively. The determinate five-year survival rates (41-43%) were similar with the three irradiation regimes; mortality under five years was mainly due to cancer recurrence locally and/or distally. Incidence of recurrence with radiation-induced stage reduction (21%) was lower, especially within the pelvis, than with no stage reduction (51%). Treatment results with planned pre-operative irradiation, especially in high stage tumours, were more favourable with the shorter 2000 cGy regimen of high fractional doses than with the conventionally fractionated 4000 cGy regimen.

Adult↗

Culture-proven cytomegalovirus retinitis in a homosexual man with the acquired immunodeficiency syndrome.

A 35-year-old homosexual man with cytomegalovirus viremia developed retinitis. He also had a new syndrome consisting of a persistent T-lymphocyte deficit, pneumocystis pneumonia, recurrent Candida albicans esophagitis, skin ulcerations caused by herpes simplex virus, Type 2, disseminated Mycobacterium avium-intracellulare infection, and molluscum contagiosum. Histopathologic examination revealed bilateral necrotizing retinitis with virions in retinal, choroidal, and optic nerve tissues. Postmortem cultures of retina and vitreous were positive for cytomegalovirus.

Acquired Immunodeficiency Syndrome↗