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[Wilms tumor in hemihypertrophy].

The case of a 4-year-old boy with Wilms' tumor and hemihypertrophy is described. Wilms' tumors are frequently associated with congenital malformations of the urinary tract, with aniridia and hemihypertrophy. Hemihypertrophy is a relatively rare malformation (1:14000) in the common population, but in patients with Wilms' tumors its frequency is about 1:49. Besides Wilms' tumors tumors of the adrenal cortex and hepatoblastomas are frequently observed together with hemihypertrophy.

Arm

Congenital total hemihypertrophy and carcinoma of undescended testicle: a case report.

The first case of germinal cell tumor of the testis in a patient with congenital total hemihypertrophy is reported. The literature is discussed with emphasis placed on the frequent association of hemihypertrophy with oncogenesis and teratogenesis. We conclude that because of the high incidence of malignancy in the undescended testis prophylactic orchiectomy should be considered in a patient with hemihypertrophy and cryptorchidism.

Cryptorchidism

The role of genetic factors in the etiology of Wilms' tumor: two pairs of monozygous twins with congenital abnormalities (aniridia; hemihypertrophy) and discordance for Wilms' tumor.

Wilms' tumor was diagnosed in two children each of whom has an identical twin. In one of the pairs of twins the aniridia syndrome with psychomotor retardation was present in both children, but Wilms' tumor was found in only one. In the other twins hemihypertrophy as well as Wilms' tumor were identified in one child, whereas neither of these abnormalities was present in her twin sister. These findings lend support to a hypothesis that the development of Wilms' tumor requires the occurrence of two successive mutational events, one of which may be a germinal mutation. The presence of aniridia, hemihypertrophy, or other associated congenital abnormalities may aid in distinguishing between hereditary and sporadic forms of Wilms' tumor.

Abnormalities, Multiple

Congenital hemihypertrophy and malignant giant pheochromocytoma - a previously undescribed coincidence.

This is apparently the first report on connatal hemihypertrophy with malignant pheochromocytoma. The coincidence of hemihypertrophy with other diseases, particularly neuroectodermal dysplasias on the one hand and the frequent association of neuroectodermal dysplasias with pheochromocytoma on the other, are emphasized. Furthermore, basically known particularities of this case as malignancy of the tumor, the unusual size of the tumor in children, and the normal catecholamine levels in serum as well as the normal excretion of vanillylmandelic acid are discussed.

Adolescent

Infantile hepatic hemangioendotheliomas associated with hemihypertrophy.

An infant presented with congenital hemihypertrophy, hepatomegaly, and a low thoracic paraspinal mass. Liver scan showed multiple defects in the uptake of radioisotope. Surgical exploration revealed multiple infantile hemangioendotheliomas of the liver and a paraspinal hemangioendothelioma. In review of 69 reported cases of infantile hemangioendothelioma of the liver and viscera, there has been no previous note of hemihypertrophy associated with this abnormality.

Female

Hemihypertrophy in a boy with renal polycystic disease: varied patterns of presentation of renal polycystic disease in his family.

Hemihypertrophy is a condition that has been described in association with a variety of other malformations and diseases; quite often these have had a renal origin. -It is the purpose of this paper to record and discuss the occurrence of polycystic disease in a family, to note that the disease was manifest as either the "adult" or the "infantile" form, and detail the fact that one member of the family who had infantile type polycystic disease also had hemihypertrophy. -The pattern of inheritance of renal polycystic disease, its age at presentation and the variation in the boy of its manifestation with the passage of time are discussed. The need for caution in offering a prognosis and genetic counselling is stressed.

Child

Hemihypertrophy with unilateral folliculitis and acne.

A case of congenital hemihypertrophy, right-sided folliculitis and acne in a 16-year-old boy is described. Except for hemihypertrophy and skin changes on the right side, no other abnormalities were found. A similar combination could not be found in the literature.

Acne Vulgaris

Idiopathic congenital hemihypertrophy with associated ipsilateral benign nephromegaly.

Two cases of idiopathic congenital hemihypertrophy with ipsilateral benign nephromegaly are presented. Excretory urography usually differentiates benign renal enlargement from Wilm's tumor in these patients. Angiography is indicated in equivocal cases. The clinical features, differential diagnosis, associated conditions, and importance of radiologic reevaluation in patients with hemihypertrophy are discussed.

Angiography

[Hemihypertrophy].

Together with a casuistic description of a hemihypertrophy partialis cruciata vera congenita of a 20-year-old male patient a survey of literature is given concerning the problem of the hemihypertrophy.

Adult

Wilms' tumor and adrenocortical carcinoma with hemihypertrophy and hamartomas.

A girl with hemihypertrophy and hamartomas, now 14 years old, had Wilms' tumor and subsequently developed adrenocortical carcinoma. The occurrence of the two tumors with the signs of an inborn defect of growth control supports the hypothesis that both tumors can be caused by the same etiologic factors, which are also teratogenic. An alternative explanation of induction of the second tumor by previous radio- and chemotherapy is discussed. Possible relationships between our case and the syndrome of Wiedemann and Beckwith are pointed out.

Adolescent

Hemihypertrophy of the human corpus cavernosum.

Hemihypertrophy of the right corpus cavernosum and its accompanying thickened tunica albuginea are responsible for the left lateral deviation in congenital curvature of the penis. Surgical correction is more predictable when the lengths of the dorsal and ventral curvatures of the corpora cavernosa are known.

Adolescent

Congenital asymmetry (hemihypertrophy) and abdominal disease: radiological features in 9 cases.

Coexistent abdominal disease was found in 9 cases of congenital asymmetry. These patients appear to be at risk of malignant neoplasms of the adrenal gland, kidney, and liver. Five of the 6 neoplasms in this group of patients were malignant. Approximately 25% of the reported cases of hemihypertrophy have been associated with hamartomas or congenital defects, especially genitourinary anomalies. Benign disorders encountered in this group included medullary sponge kidney, renal ectopia, renal cyst, nephromegaly, adrenomegaly, and hypospadius.

Abdomen

[Hemihypertrophy and functioning adrenal tumor (author's transl)].

The case of a secreting adrenal tumor in a seven month old hemihypertrophic girl is described. Some features of hemihypertrophy and its relationships with the E.M.G. Syndrome and abdominal tumors are commented upon. The reported case is a typical association of malformation with tumor and recalls the close relationship between teratogenesis and oncogenesis.

Adenoma