Carbonic Anhydrase Type II Deficiency Syndrome

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منابع مشابه

The neurology of carbonic anhydrase type II deficiency syndrome.

Carbonic anhydrase type II deficiency syndrome is an uncommon autosomal recessive disease with cardinal features including osteopetrosis, renal tubular acidosis and brain calcifications. We describe the neurological, neuro-ophthalmological and neuroradiological features of 23 individuals (10 males, 13 females; ages at final examination 2-29 years) from 10 unrelated consanguineous families with ...

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Carbonic anhydrase II deficiency a novel mutation.

Carbonic anhydrase II (CA II) deficiency is an extremely rare autosomal recessive disorder, characterised by a triad of osteopetrosis, renal tubular acidosis and cerebral calcifications. A 12 year old boy with classical features of CA II deficiency is reported who was found to be homozygous for the mutation in CA II gene and parents were heterozygous for the same mutation .To the best of our kn...

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Carbonic Anhydrase II Deficiency in a Saudi Woman

OBJECTIVE Carbonic anhydrase (CA) II deficiency is a rare autosomal recessive disorder caused by mutation in the CA II gene that leads to osteopetrosis, renal tubular acidosis (RTA), and cerebral calcification. Our aim is to present a patient with the classic triad of CA II deficiency syndrome to enhance the awareness about this rare syndrome. METHODS We describe the clinical and radiological...

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Renal Tubular Acidosis, Osteopetrosis, and Cerebral Calcification: A Rare Syndrome Caused by Carbonic Anhydrase II Deficiency

A 7‐year, 10‐month‐old boy, product of a consanguineous marriage, presented with short stature and a recent fracture of left forearm following a trivial trauma. There was no significant family history. His height was 103 cm (−3.7 standard deviation) [Table 1]. He had dysmorphic facies such as prominent forehead, thick lower lip, and blue sclera. He had 24 teeth; his temporary teeth had not shed...

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Carbonic anhydrase II deficiency syndrome--clinico-pathological, biochemical and molecular studies.

We reported on three unrelated Japanese families with carbonic anhydrase II (CA II) deficiency syndrome. In the present study, the CA II gene was sequenced in the family of a patient with hybrid type renal tubular acidosis whose parents were nonconsanguineous, and a T to G transition at exon 2 was identified. The change results in the substitution of the stop codon (TAG) at position 40 for Tyr ...

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ژورنال

عنوان ژورنال: Pediatric Neurology Briefs

سال: 2012

ISSN: 2166-6482,1043-3155

DOI: 10.15844/pedneurbriefs-26-1-8