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Biomedical subjects

C H Klippel

Publications and source records attributed to C H Klippel.

8 recordsLinked to original sources

Intractable diarrhea in children with VIP-secreting ganglioneuroblastomas.

This paper describes two children with diarrhea, hypokalemia, and VIP-secreting ganglioneuroblastomas, and includes further evidence that VIP is the cause of the loose stools among patients with such tumors. It suggests that the level of this peptide should be measured in pediatric patients with intractable diarrhea of unknown etiology. Clinicians have long recognized intractable diarrhea as an occasional feature of neuroblastoma, ganglioneuroblastoma or ganglioneuroma. Until recently catecholamine was thought to cause the frequent stools in some patients with these tumors. New radioimmunoassay methods, the identification of new hormones and hormone-like substances, and the discovery that some of the tumors which cause diarrhea secrete Vasoactive Intestinal Peptide (VIP), make it probable that this substance is responsible for the diarrhea in such patients.

Child, Preschool↗

Surgical implications of pediatric endocrine disorders.

The adrenal gland and the gonads both originate in the embryonic posterior mesenchyme and secrete steroids. Malfunction in either of them results in certain typical developmental problems. The adrenogenital syndrome resulting from excess androgen production occurs as masculinization in the female and precocious maturation in the male. Adrenal tumor usually has the same presentation; feminizing tumors are rare and usually are malignant. Gonadal problems include enlargement of sex organs, ambiguous genitalia, missing testes, and tumor causing precocious puberty. Endocrine problems must be assessed as early as quickly as possible, particularly cases of ambiguous genitalia, so that a proper sex assignment can be made and treatment instituted before parental attitudes arise that will affect the child later on. Surgical restructuring or removal of unneeded organs often is necessary.

Adrenal Gland Neoplasms↗

Pyloric atresia and epidermolysis bullosa letalis: a lethal combination in two premature newborn siblings.

Pyloric atresia and epidermolysis bullosa letalis are rare congenital anomalies. The association of the two diseases in siblings has not been reported previously. This paper describes such as association in two newborn infants of caucasian parents with no family history of similar anomalies. Problems in management included severe fluid and electrolyte losses, systemic infection, and prematurity, which led to the death of the two infants. The question is raised whether the two abnormalities are expressions of closely linked genes. The presence of epidermolysis bullosa should alert the pediatrician to the possiblity of a coexisting pyloric atresia.

Epidermolysis Bullosa↗

Electrosurgical skin incision.

A controlled clinical study shows that there is little difference between skin incisions made with a scalpel or by electrosurgical instruments.

Adolescent↗