Fetal adrenal cytomegaly.

Gau, Gillian; Bennett, Michael J.

doi:10.1136/jcp.32.3.305

Cited by 4 publications

(3 citation statements)

References 7 publications

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“…Aterman (1, 10) suggested that the giant nuclei of cytomegalic cells were polyploid; they were later found to contain up to 25 times the normal amount of DNA (9). At a time when the transient zone is normally largely degenerated, DNA synthesis was still occurring in the cytomegalic nuclei (Ki-67 positivity) in our patients as late as 2 months postpartum,.…”

Section: Discussionmentioning

confidence: 99%

“…These cells each had a very large, round, hyperchromatic nucleus and cytoplasm more eosinophilic than that of the transient cortex cells; they stood out because of their great size. Later, these cells were found elsewhere—in adult patients with congenital adrenal hypoplasia (10), in newborns with congenital anomalies (Beckwith-Wiedemann syndrome) (2 Beckwith, 1969), in newborns with Rh incompatibility (1) and, very rarely, in healthy adults (23). None of these patients had Cushing syndrome.…”

Section: Introductionmentioning

confidence: 99%

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Massive Neonatal Adrenal Enlargement Due to Cytomegaly, Persistence of the Transient Cortex, and Hyperplasia of the Permanent Cortex

Carney

Kitsuda

et al. 2012

American Journal of Surgical Pathology

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We describe massive enlargement of both adrenal glands in 3 newborns, 2 girls and 1 boy. Two had hemihypertrophy and other congenital abnormalities but no identified genetic mutation; the third had genetically proven Beckwith-Wiedemann syndrome. Two had severe Cushing syndrome, the third had hypercortisolemia but no clinical Cushing syndrome. Bilateral adrenalectomy cured Cushing syndrome in the 2 with the severe symptoms; total adrenal weight was 44 and 53 gm in the patients, respectively. Unilateral adrenalectomy was performed in the third patient: the gland weighed 52 g; postoperatively, the patient's hypercortisolemia normalized and, concomitantly, the enlarged contralateral adrenal gland had a 5-fold decrease in size to slight enlargement 6 years postoperatively. Microscopically, the 3 patients had similar pathology: massive adrenal enlargement due to a combination of cytomegaly, persistence of the transient cortex, and hyperplasia of the permanent cortex. The pathological findings were most likely the result of the genetic mutation identified in 1 patient and of an unknown mutation in the remaining 2 patients.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Massive Neonatal Adrenal Enlargement Due to Cytomegaly, Persistence of the Transient Cortex, and Hyperplasia of the Permanent Cortex

Carney

Kitsuda

et al. 2012

American Journal of Surgical Pathology

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“…Adrenocortical cytomegaly has been reported in fetuses with congenital rubella syndrome and mumps [1,6], but no viruses have been identified. Although AC can be an incidental finding, it is known to be associated with specific conditions in the fetal and neonatal population, in particular certain developmental anomalies and Rh incompatibility [1,6,9–11]. Rhesus incompatibility, manifesting as immune hydrops fetalis, is the condition in which AC is commonly found, with up to 69% of cases in some series [1,5–7].…”

Section: Discussionmentioning

confidence: 99%

Adrenal Cytomegaly is a Frequent Pathologic Finding in Hemoglobin Bart Hydrops Fetalis

2012

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Adrenocortical cytomegaly (AC) is a relatively uncommon phenomenon but tends to occur in certain situations, including specific congenital anomalies and hydrops due to maternal-fetal Rhesus incompatibility. Because the pathology in the latter condition does not differ greatly from hemoglobin (Hb) Bart hydrops fetalis, we performed a retrospective review of fetal and perinatal autopsy cases with Hb Bart to determine the prevalence of AC in that condition. Over a 10-year period (2001-2010) at King Chulalongkorn Memorial Hospital, there were 16 hydropic cases confirmed to have Hb Bart. Adrenocortical cytomegaly was found in 13 cases (81%). For comparison, we determined the occurrence of AC in cases of hydrops fetalis not due to Hb Bart (n = 33) and a heterogeneous group of congenital anomalies (n = 34). Adrenocortical cytomegaly was identified in only 1 case of Beckwith-Wiedemann syndrome and 2 cases of anencephaly. Thus, AC is a common finding in cases of Hb Bart, a finding not previously documented. Moreover, our study suggests that Hb Bart is one of the conditions most commonly associated with AC. The reasons for this are not known. The mean Hb levels for the hydrops cases with Hb Bart and those with other forms of anemia showed no significant difference (P = 0.63), nor was there any significant difference in Hb levels between cases of Hb Bart with and without AC. Nonetheless, the consistency of AC in cases of Hb Bart suggests that further study of this particular group of patients might shed light on the pathogenesis of this poorly understood pathologic finding.

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Adrenal Cytomegaly

Nosé¹

2018

Diagnostic Pathology: Endocrine

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Fetal adrenal cytomegaly.

Cited by 4 publications

References 7 publications

Massive Neonatal Adrenal Enlargement Due to Cytomegaly, Persistence of the Transient Cortex, and Hyperplasia of the Permanent Cortex

Massive Neonatal Adrenal Enlargement Due to Cytomegaly, Persistence of the Transient Cortex, and Hyperplasia of the Permanent Cortex

Adrenal Cytomegaly is a Frequent Pathologic Finding in Hemoglobin Bart Hydrops Fetalis

Adrenal Cytomegaly

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