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Biomedical subjects

Najeeba Riyaz

Publications and source records attributed to Najeeba Riyaz.

5 recordsLinked to original sources

Hemihypertrophy and primary small intestinal lymphangiectasia in incontinentia pigmenti achromians.

A rare occurrence of primary small intestinal lymphangiectasia in an 8-year-old girl with incontinentia pigmenti achromians is reported. In addition, she had right sided hemihypertrophy. Though intestinal lymphangiectasia is known to have a few syndromic associations, its co-existence in a child with incontinentia pigmenti achromians has not yet been reported in the literature. Hemihypertrophy is also extremely rare in the latter and only very few instances of an association between the two have been documented previously.

Body Constitution↗

Focal dermal hypoplasia (Goltz syndrome).

A 7-year-old girl born of non-consanguineous marriage was evaluated for facial dysmorphism. She had multiple skeletal anomalies like hypoplasia of the right mandible, narrow nasal bridge with broad tip and unilateral notching of the right ala nasi, concomitant squint and low set ears. She also had generalized hypopigmented, atrophic linear macules, multiple papillomas, fat herniations, umbilical hernia, hypoplastic nails, cicatricial alopecia, mild mental retardation, 'lobster-claw' hand and osteopathia striata of long bones, pointing to a diagnosis of Goltz syndrome. The unusual features noted were absence of the left first rib and aortic regurgitation.

Abnormalities, Multiple↗

Rosai-Dorfman syndrome.

Rosai-Dorfman syndrome is characterized by sinus histiocytosis with massive lymphadenopathy. A 25-year-old woman presented with multiple erythematous and yellowish papules on the forehead, cheeks, chin and thigh. She had massive generalized, firm, non-tender and non-matted lymphadenopathy and mild hepatomegaly. Her hemogram was normal. A skin biopsy showed collections of histiocytes with emperipolesis and plenty of plasma cells. A lymph node biopsy showed partial loss of architecture, dilated sinuses filled with histiocytes, which showed lymphocytophagocytosis, anisonucleosis and a clear cytoplasm. She was referred to an oncologist for further management.

Adult↗

Poland syndrome (anomaly) with congenital hemangioma: a new association.

Unilateral defect of pectoral muscle and ipsilateral syndactyly constitute Poland syndrome. Absence or hypoplasia of the breast and nipple, axillary hair loss and dermatoglyphic abnormalities have also been reported in this syndrome. The primary defect could be in the development of the proximal subclavian artery with early deficit of blood flow to the distal limb and the pectoral region, resulting in partial loss of tissue in those regions. However, the association of congenital hemangioma with Poland sequence has not been observed so far. Such an association is being reported here in a 1-year-old infant, second-born of nonconsanguineous parents, who also had polydactyly instead of the documented syndactyly.

Arm↗