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Early onset of tuberous sclerosis with chylous ascites: a case report.

Dalili H, Amini E, Akbari Asbagh P, Esmaeilnia Shrivany T, Niknafs N, Nayyeri F, Shariat M, Talebian S, Akbari Asbagh N, Ghorban Sabagh V - Iran. J. Public Health (2015)

Bottom Line: This report summarizes a case of TSC in a premature infant, born at 34 weeks' gestation with ascites.After birth, multiple cardiac mass, subependymal cysts and hypopigmented macules were detected.To our knowledge, this is the first case report of early onset of TSC with chylous ascites in Iran.

View Article: PubMed Central - PubMed

Affiliation: Family Health Institute, Breastfeeding Research Center, Tehran University of Medical Sciences, Tehran, Iran.

ABSTRACT

Background: Tuberous Sclerosis Complex (TSC) is an autosomal-dominant hereditary disorder. This syndrome is characterized by tumor-like malformations in several organs, as well as the heart. This report summarizes a case of TSC in a premature infant, born at 34 weeks' gestation with ascites. After birth, multiple cardiac mass, subependymal cysts and hypopigmented macules were detected. To our knowledge, this is the first case report of early onset of TSC with chylous ascites in Iran.

No MeSH data available.


Related in: MedlinePlus

Subepandymal cyst
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Figure 4: Subepandymal cyst

Mentions: Another paracentesis was repeated in the next day and 70 ml of ascitic fluid were removed. Screening tests (e.g., cell count and differential, albumin and total protein concentration) were performed on the initial specimen, which was negative for malignant cells, bacterial culture and biochemical test. Serological testing for maternal TORCH, human immunodeficiency virus and bacterial cultures of blood, urine and endocervical swabs were negative. Further sonographic and MRI examinations detected subependymal cysts on the right and left sides (Fig. 4). In addition, neonatal echocardiography showed multiple cardiac masses in the left and right ventricle (Fig. 5).There was no need to perform any heart medications because the heart appeared hemodynamically stable. The findings of hydrops and cardiac tumors were consistent with tuberous sclerosis. On follow up, the infant showed hypopigmented macules on the skin (Fig. 6) and jerky movements. Both parents underwent several evaluations for TSC, including ophthalmological examination, skin examination, echocardiogram, abdominal ultrasound scan and brain CT scan, all of which have been normal.


Early onset of tuberous sclerosis with chylous ascites: a case report.

Dalili H, Amini E, Akbari Asbagh P, Esmaeilnia Shrivany T, Niknafs N, Nayyeri F, Shariat M, Talebian S, Akbari Asbagh N, Ghorban Sabagh V - Iran. J. Public Health (2015)

Subepandymal cyst
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4402421&req=5

Figure 4: Subepandymal cyst
Mentions: Another paracentesis was repeated in the next day and 70 ml of ascitic fluid were removed. Screening tests (e.g., cell count and differential, albumin and total protein concentration) were performed on the initial specimen, which was negative for malignant cells, bacterial culture and biochemical test. Serological testing for maternal TORCH, human immunodeficiency virus and bacterial cultures of blood, urine and endocervical swabs were negative. Further sonographic and MRI examinations detected subependymal cysts on the right and left sides (Fig. 4). In addition, neonatal echocardiography showed multiple cardiac masses in the left and right ventricle (Fig. 5).There was no need to perform any heart medications because the heart appeared hemodynamically stable. The findings of hydrops and cardiac tumors were consistent with tuberous sclerosis. On follow up, the infant showed hypopigmented macules on the skin (Fig. 6) and jerky movements. Both parents underwent several evaluations for TSC, including ophthalmological examination, skin examination, echocardiogram, abdominal ultrasound scan and brain CT scan, all of which have been normal.

Bottom Line: This report summarizes a case of TSC in a premature infant, born at 34 weeks' gestation with ascites.After birth, multiple cardiac mass, subependymal cysts and hypopigmented macules were detected.To our knowledge, this is the first case report of early onset of TSC with chylous ascites in Iran.

View Article: PubMed Central - PubMed

Affiliation: Family Health Institute, Breastfeeding Research Center, Tehran University of Medical Sciences, Tehran, Iran.

ABSTRACT

Background: Tuberous Sclerosis Complex (TSC) is an autosomal-dominant hereditary disorder. This syndrome is characterized by tumor-like malformations in several organs, as well as the heart. This report summarizes a case of TSC in a premature infant, born at 34 weeks' gestation with ascites. After birth, multiple cardiac mass, subependymal cysts and hypopigmented macules were detected. To our knowledge, this is the first case report of early onset of TSC with chylous ascites in Iran.

No MeSH data available.


Related in: MedlinePlus