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Biomedical subjects

K Sekido

Publications and source records attributed to K Sekido.

24 records · Page 2Linked to original sources

[Controlling precocious puberty--surgical excision of hypothalamic hamartoma causing precocious puberty].

Among the causes of precocious puberty, hypothalamic hamartoma comprises a small percentage. However, the frequency of precocious puberty in the presence of hypothalamic hamartoma is quite high. Recently, results of surgery in 14 cases of hypothalamic hamartoma were reported. Precocious puberty completely subsided in three cases and slight improvement was achieved in another three cases. We performed surgery in four patients with hypothalamic hamartomas, with the goal of decreasing the symptoms of precocious puberty. The patients were two females (aged 1 yr, 3 mo and 6 mo) and two males (aged 3 yr, 7 mo and 1 yr, 9 mo). The main symptoms were precocious puberty and mental retardation of varying degrees. The males had excessive growth of body and external genitalia, while the females had genital bleeding and premature breast development. In each case, computed tomographic scans disclosed a round, isodense mass in the interpeduncular cistern, attached to the base of the hypothalamus. Contrast enhancement was negative. Endocrinologically, in case 1, testosterone was 92.6 ng/ml, FSH was 16 mIU/ml, and LH was 2.2 mIU/ml. Although LH was within normal limits, it overresponded to LH-RH stimulation. In case 2, estrogen was 13.5 ng/day, LH was 5.2 mIU/ml, FSH was 5.3 mIU/ml, and LH showed an exaggerated response to LH-RH stimulation. In case 3, testosterone was 362 ng/ml, LH was 8.8 mIU/ml, FSH was 4.8 mIU/ml, and LH showed an abnormally high response to LH-RH stimulation. In case 4, LH was 18.4 mIU/ml, FSH was 12.0 mIU/ml, and both hormones were stimulated abnormally strongly by LH-RH.(ABSTRACT TRUNCATED AT 250 WORDS)

Child, Preschool↗

Hypothalamic hamartoma successfully treated by operation. Case report.

An 18-month-old boy was diagnosed as having a hypothalamic hamartoma. When he was 1 year old, he developed precocious puberty, and at 18 months old, endocrinological tests revealed abnormally high follicle-stimulating hormone, luteinizing hormone, and testosterone levels. The center of the hamartoma was subtotally excised, as confirmed on the postoperative computerized tomography scan. Precocious puberty subsided after the operation.

Brain Neoplasms↗

A cloverleaf skull syndrome probably of Beare-Stevenson type associated with Chiari malformation.

A case of cloverleaf skull (CLS) syndrome with Chiari malformation was reported. The patient developed congenital hydrocephalus, upper airway obstruction and breath holding spells. Ventriculo-peritoneal shunt improved the hydrocephalus, but the patient progressively developed apneic episodes in spite of intubation. Brain magnetic resonance imaging (MRI) disclosed severe Chiari malformation. Laminectomy of the cervical vertebrae and craniectomy at the occipital bone were performed to relieve the brain stem compression. Postoperatively, the respiratory symptoms improved slightly, but 8 weeks later the patient suddenly died. Literature survey revealed that this is the second case report of CLS associated with Chiari malformation. It was assumed that the respiratory problems in our patient are ascribable to the Chiari malformation as well as the upper airway obstruction. The patient here reported seems to be a case of Beare-Stevenson cutis gyrata syndrome, in view of an association of multiple characteristic dysmorphic features, in addition to CLS.

Abnormalities, Multiple↗

Cytologic features of desmoplastic infantile ganglioglioma: a report of two cases.

BACKGROUND: Desmoplastic infantile ganglioglioma (DIG) is a rare intracranial tumor of infancy, characterized by solid and cystic components, voluminous size and supratentorial location. Although its histologic features have been reported, there has been no cytologic description of the tumor. Cytologic findings on imprint and aspirated material from two cases of histologically verified DIG are discussed. CASES: A 12-month-old male with cutis marmorata telangiectatica congenita and a 44-month-old female with episodes of spasm were referred to our center. Radiologic examination revealed a large, cystic, supratentorial mass in both patients. The mass was surgically removed, and histology revealed prominent desmoplasia with a mixture of astroglial and neuronal cells. Cytologic findings on imprint material and the needle aspirates taken from the cystic area of the tumor revealed a few isolated or sheetlike arrangements of small cells positive for glial fibrillary acidic protein and a few large cells with abundant cytoplasm and prominent nucleoli, positive for neuronal marker. CONCLUSION: Although distinction between ganglioglioma and DIG may be difficult by cytology alone, with the characteristic clinical presentation and radiologic findings, the possibility of DIG should be kept in mind when the specimens contain both astroglial and neuronal elements.

Biopsy, Needle↗