Postmortem findings in the lungs of two siblings with nonimmune hydrops and lung hypoplasia are presented. In one, a girl, the hypoplasia was severe, with reduced numbers of airway generations and markedly reduced radial alveolar counts. In the other, a boy, the hypoplasia of both airway and alveolar development was mild. The reason for the difference in the growth of the lungs in these two chi...

Relapsing polychondritis (RP) is a rare multisystem disorder. Myelodysplastic syndrome (MDS) with erythroid hypoplasia/aplasia is a rare form of myelodysplasia. Several cases of RP associated with MDS have recently been described. However, RP associated with MDS with erythroid hypoplasia/aplasia has never been reported. There was only one case report of polymyalgia rheumatica associated with MD...

6. Aoyama M., Sawada h., Shintani Y., Isomura I., Morita A. Case of unilateral focal dermal hypoplasia (Goltz syndrome). J Dermatol. 2008; 35:33-5. 7. Leoyklang P., Suphapeetiporn K., Wananukul S., Shotelersuk V. Three novel mutations in the POrCn gene underlying focal dermal hypoplasia. Clin Genet. 2008; 73:373-9. 8. Goltz r.W. Focal dermal hypoplasia syndrome. An update. Arch Dermatol. 1992; ...

Enamel hypoplasia is an important clinical problem commonly seen in children born to diabetic women. We aimed to characterize the enamel hypoplasia in Wistar rats born to alloxan-induced diabetes mellitus rats. Groups consisted of pregnant rats supplemented (ISDR) or not (NISDR) with insulin and controls, in which sterile saline solution was administered instead of alloxan or insulin. The mandi...

Lung tissue developmental abnormalities are seldom reported. According to the classification of Schneider (1900), which was amended by Boyden (1955), they include pulmonary agenesis, aplasia and hypoplasia. Due to the early onset of symptoms, lung agenesis and aplasia are usually detected soon after birth. Conversely, lung or lobar hypoplasia may remain clinically silent for a long time. A sing...

OBJECTIVES To study the epidemiology of optic nerve hypoplasia. DESIGN AND METHODS Children with optic nerve hypoplasia and visual impairment were identified through the Swedish Register of Visually Impaired Children. Pre- and perinatal characteristics were obtained from the Medical Birth Registry and by scrutinizing pregnancy and delivery records. Clinical characteristics of children with op...

Maxillary hypoplasia that necessitates surgical advancement affects approximately 25% of patients born with cleft lip and palate. Syndromic conditions such as Crouzon may also be accompanied by significant maxillary hypoplasia. Severe maxillary hypoplasia can result in airway obstruction, malocclusion, proptosis, and facial disfigurement. For optimal stability, severe hypoplasia is best address...

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We present a child with the rare association of ct ngenital muscular dystrophy, hypoplasia of the lateral abdo ninal wall musculature and hypoplasia of the external genitc lia.

BACKGROUND: Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a rare disorder described as aplasia or hypoplasia of uterus and vagina due to an early arrest in the development Mullerian ducts. The etiology MRKH remains uncertain: Although at beginning, it was mentioned that this result sporadic abnormalities. It has recently been assumed genetic background cause increasing number familial cases...