Normal Male Infant Research Articles

Androluteoma syndrome of pregnancy

The clinical course of a 26-year-old primigravid woman with a virilizing luteoma of pregnancy is described. It was demonstrated by endocrine studies that the tumor secreted an excessive amount of testosterone. After removal of the luteoma in week 32 of pregnancy, the virilizing symptoms of the mother completely regressed. A normal male infant was delivered in week 39. From 100 luteomas of pregnancy described in the literature, 26 cases have been cited to elicit endocrine virilizing activity. An evaluative synopsis indicates that including the case described in this paper only 13 can be regarded as a clinical, morphologic, and functional entity. This entity should be differentiated from the inhomogeneous group of luteomas as the “androluteoma syndrome of pregnancy.” It is characterized by the following major criteria: virilization, beginning with the second trimester of pregnancy; histologic criteria of a luteoma of pregnancy; production of testosterone, leading to excessive plasma levels (exceeding the concentration of androstenedione), and masculinization of female fetuses.

Persistent Mullerian Structures in a Male Neonate

A 2-week-old male infant was diagnosed as having persistent Mullerian structures. Microscopically normal testes were found, as well as a uterus, cervix, and fallopian tubes. Plasma testosterone values responded normally to human chorionic gonadotrophin stimulation. This condition at times is familial, and genetic counseling is indicated. CASE REPORT A 2-week-old white boy presented with a history of a mass in the left groin. This mass was not present at birth. There is no family history of inguinal mass and the patient has no siblings. Physical examination revealed a normal male infant with a 2-cm to 3-cm hard mass in the left inguinal area.

Normal Gestation and Diminished Androgen Responsiveness in an Untreated Patient with 21-Hydroxylase Deficiency

A 22-year-old woman with 21-hydroxylase deficiency but minimal clinical evidence of androgenization was studied. She had conceived twice and had borne a normal male infant without therapy of any kind. The diagnosis of 21-hydroxylase deficiency was substantiated by the findings of 17-ketosteroid and pregnanetriol excretions of 18.1 and 8.1 mg/24 hours, respectively. Adequate basal compensation was indicated by a fasting plasma cortisol of 17.5 mug/dl. Plasma ACTH (207 pg/ml), testosterone (216 ng/dl) delta4-androstenedione (649 mg/dl), progesterone (249 ng/dl) and 17alpha-hydroxyprogesterone (4820 ng/dl) were all significantly elevated.

Ablatio penis: normal male infant sex-reassigned as a girl.

Forty-five cases of genetic males were assigned and habilitated as females, 43 because of a congenitally defective penis (micropenis with or without hypospadias), and two because of infantile ablatio penis. One of the latter has an identical twin brother as a control. Now 9 years old, she has differentiated a female gender identity in marked contrast to the male gender identity of her brother. Some of the other patients are now adolescent or adult in age. They demonstrate that the twin can expect to be feminine in erotic expression and sexual life. Maintained on estrogen therapy, she will have normal feminine physique and a sexually attractive appearance. She will be able to establish motherhood by adoption.

Virilization in pregnancy associated with an ovarian cystadenoma

A teen-age patient, who became virilized in the course of pregnancy is described. The virilizing syndrome was due to a serous cystadenoma of the ovary. A marked difference in plasma testosterone concentration between specimens obtained preoperatively and postoperatively was demonstrated, indicating testosterone secretion by the neoplasm. Virilization regressed completely by the sixth postoperative month. The patient became pregnant one year after tumor resection and delivered a normal male infant. This report represents the first serous cystadenoma associated with virilization in pregnancy. The literature on the subject is reviewed.

Bromoderma in pregnancy.

A 33-year-old white woman, gravida 6, para 5, abortus 0, developed bromoderma during the sixth month of her pregnancy. She had ingested approximately 30 capsules of Nervine® per day for the previous three months. She responded to treatment and a normal male infant was born at term.

Screening for bacteriuria in the newborn nursery: Collection of the suitable urine specimen

Summary A program for daily newborn screeningfor bacteriuria was instituted in the nursery of a community hospital. One individual, a laboratory technician, was assigned the daily task of applying a two-chambered collector (U-Bag) to the grossly clean perineum of the normal male or female fullterm newborn infant. The collector was monitored until the infant voided but was removed at the end of one hour and discarded if the child failed to void. Quantitative cultures were done by the same technician in the nursery. Voided specimens suitable for culture and analysis were obtained from 81 per cent of the study population. Bacterial counts exceeded 10 5 organisms per milliliter in only 6 per cent of the specimens collected; in no instance dis this degree of bacteriuria persist in three consecutive specimens.

Depressed immunoglobulin G in newborn infants with Down's syndrome

Serum immunoglobulins (IgG, IgM, and IgA) were quantitated in samples from 32 newborn infants with Down's syndrome (trisomy 21). When compared with the values of normal newborn infants, IgG levels in the infants with trisomy 21 were significantly lower. No evidence for diminished maternal levels of IgG were found. By using Gm and Inv markers it was found that the fetus with Down's syndrome produced IgG. This fetally produced IgG was not proportionately greater in amount than that found in normal fetuses. A low serum IgG value was observed in one newborn infant with mongolism who had a D/G translocation. Another infant with trisomy 21 was a female twin of a normal male infant; the normal member of the twin pair did not have the depressed serum IgG value observed in the twin with mongolism.

Spontaneous Sexual Development in a Chromatin Negative Female with XO Blood Leukocyte Karyotype

Although Turner's syndrome is usually associated with a spectrum of phenotypic abnormalities, the relatively constant features are short stature and the absence of breast development, menstruation, and fertility. An unusual exception to this general prognosis was the case reported by Bahner, et al.1,2 Their patient had short stature and a low occipital hairline; she was chromatin negative and karyotypically XO in cultures of bone marrow, skin, and both ovaries. She showed normal sexual maturation and at age 31 years delivered a normal male infant. The present report describes a chromatin negative patient who exhibited spontaneous breast development at age 12 years. This prompted a histologic and chromosomal investigation, the results of which are discussed below.

An intestinal polyp of the umbilical cord.

TUMORS of the umbilical cord are rare. 1,2 It is, therefore, of interest to record the case of a grossly visible, pedunculated polyp of the umbilical cord which, on histological examination, was found to consist of well-formed intestine. Gross Findings A white primigravida, aged 21 years, gave birth at term to a normal male infant weighing 2,949 gm (6 lb 8 oz). The placenta (425 gm) and the membranes, received in 10% formaldehyde solution fixative, did not appear remarkable. The umbilical cord, 40 cm long and 0.8 cm wide, presented three vessels and seemed normal, but about 5 cm from the cut end there was seen, on the surface of the cord, a distinct nodule. It was oval, measured about 0.5 cm in its longer axis and appeared grayish-tan with a glistening, smooth surface. The nodule was freely movable on the surface of the cord, to which it apparently was

Congenital aortic stenosis in pregnancy, corrected by extracorporeal circulation, offering a viable male infant at term but with anomalies eventuating in his death at four months of age--report of a case.

Severe heart disease associated with an unusual combination of circumstances necessitated surgical correction of congenital aortic stenosis in a 4-months pregnant, 23-year-old housewife. Extracorporeal circulation was utilized to support the mother and fetus during the 20minute period of arrested circulation to the heart. Following this successful surgery and an uneventful postoperative convalescence, pregnancy, and labor, a full-term viable infant was delivered. The child had multiple congenital anomalies and succumbed at the age of 4 months. Fourteen months later, a normal male infant was delivered from the same mother. Despite any proof of cause and effect in this situation, it appears wisest to assume, until proved otherwise, that the congenital anomalies in the first infant appeared as a direct result of the perfusion.

Aortic dissection in pregnancy: A case of Marfan's syndrome

1. 1. A review of the literature on aortic dissection is presented, emphasizing the different etiologic and pathogenetic factors involved and their specific histologic features. Aortic dissection in the young individual is characterized by an early degeneration of the elastic tissue components of the aortic medial layer, as is aortic dissection in pregnancy and Marfan's syndrome. Following the degeneration of the elastic laminae, secondary changes are observed in the smooth muscle tissue, with ingrowth of numerous thin-walled vessels and development of cystic mucin-filled spaces, whereby the aortic wall becomes markedly weakened and susceptible to dissection. 2. 2. A case of Marfan's syndrome with aortic dissection during pregnancy is presented exhibiting less elastic tissue bulk in the aorta than normally seen. The question is raised as to whether an absolute decrease in elastic tissue mass is a constant feature in Marfan's syndrome in addition to the fragmentation of the elastic laminae previously described. 3. 3. Repeated bouts of aortic dissection were observed from the twenty-second throughout the thirty-first week of pregnancy in the present case of Marfan's syndrome, finally leading to the development of a triple-barreled aorta. Cesarean section was done electively 2 weeks before term, with delivery of a normal male infant. After delivery no further episodes of dissection were recognized. The patient expired in congestive failure 13 months after onset of dissection, which was 8 months post partum. 4. 4. We believe that gestation enhanced the aortic dissection and shortened the patient's life. It is prudent to individualize termination of pregnancy in each case, depending on the stage of gestation at which the dissection occurs. Interruption of the early pregnancy is recommended as soon as the cardiac condition is stabilized. If possible, whenever the gestation is in the third trimester, the patient should be carried to viability of the fetus and delivery by cesarean section. The authors feel that one should deviate from the usual management of obstetric care in aortic dissection and that cesarean section is the method of choice for termination of the pregnancy after viability of the fetus is assured.

Persistent Nonhemolytic Hyperbilirubinemia with Unidentified Pigment in Liver Cells Report of Case Followed in Pregnancy

Summary 1.A case of persistent nonhemolytic hyperbilirubinemia with unidentified pigment in the liver cells, first diagnosed during pregnancy and followed through delivery, is presented. 2.Pregnancy and delivery of a normal male infant were uneventful in this condition, in which the diagnosis was confirmed by transthoracic needle liver biopsy at 4 months' gestation. 3.More widespread use of liver biopsy in cases of long-standing icterus of this type will result, it is felt, in the diagnosis of a considerable number of cases of this condition.; Such early diagnosis is desirable because of the relative benignancy of this type of hyperbilirubinemia, and the possibility of avoidance of operative procedures as well as the opportunity to reassure the patient.

THE METABOLISM OF l-TYROSINE IN INFANTILE SCURVY 1

Attention has been drawn by Levine, Marples, and Gordon (1) to the relationship between ascorbic acid and the urinary excretion of aromatic amino acid intermediates by the normal premature male infant, both during periods of high protein intake and supplemental tyrosine and phenylalanine ingestion. To our knowledge, no reports have been forthcoming of similar studies with older, ascorbic acid depleted subjects.3 This communication is concerned with the metabolism of l-tyrosine in infantile scurvy. Observations have been made on the urinary excretion products, the serum content of both hydroxyphenyl compounds and amino acid nitrogen. Sealock and his co-workers (2) have demonstrated that the oral administration of l-tyrosine to scorbutic guinea pigs results in the urinary excretion of the amino acid and its two principal deaminated products, parahydroxyphenylpyruvic and parahydroxyphenyllactic acids. A proof that this metabolic disorder is primarily dependent upon the state of ascorbic acid depletion is afforded by the observation that the tyrosyluria (hydroxyphenyluria) is abolished when the vitamin is given orally or parenterally. Certain liver extracts are capable of modifying an induced tyrosyluria ( 1, 3), and recently Woodruff and Darby (4, 5) have indicated that pteroylglutamic acid (PGA) exerts a similar effect in the depleted guinea pig maintained on a basal scorbutogenic diet with supplements of l-tyrosine. By the administration of PGA to normal premature infants, Govan and Gordon (6) in several instances observed a diminution in the tyrosyluria