Myocardial calcinosis


Ten year old boy with myocardial calcinosis born from consanguineous marriage was admitted to cardiology department. At the age of 11 months he presented with the restriction of coxal joint movement. He started walking at the age of 1.5 with claudication. Joint biopsy showed granulomas and calcification {only the description given, tissue sample now unavailable}. At 5 years the boy presented with weakness, the investigation showed myocardial calcification on ECHO and calcification of aortal valve.

He was ten years old on admission to our department in may 2011. On examination the child had some changes on skin that looked like papulas or like “chicken skin”, mainly on abdomen, some in axillary crease, marked skin hyperelasticity, joint hypermobility, no restriction in coxal joint, hepatomegalia {first found at the age of two years}, frequent urination. He had a story of GI bleedings. ECHO showed massive myocardial calcinosis, EF decrease, aortal valve calcification and stenosis combined with insufficiency, LV dilatation. CT heart scan showed massive vermiform calcifications in all myocardial layers.
CT scan of chest and abdomen revealed the enlargement of internal lymph nodes. There was liver fibrosis according to liver elastometry. CT scan of head: many small petrificats in brain substance and calcification along the vessels in subcortical nuclei of both hemispheres. X-ray of hands: bone age equal to seven years. Densitometry: no osteoporosis.

Blood analyses: anemia, normal calcium blood level, parathormone - normal, low vitamin D level. Rheumathoid factor negative. High levels of circulating immune complexes.

Immunophenotype: no specific changes. Chemiluminescence of neutrophils: no specific changes. Coagulogram: mild hypocoagulation. Antibodies to toxocara, toxoplasma – negative. Antibodies to CMV – positive {both Ig G and Ig M}, PCR for CMV in urine, blood – negative.
Low calcium level in urine.
Tuberculosis, HIV, syphilis - ruled out.
Ophthalmology: no pathology. Neurology: no pathological signs.

The boy developed pneumonia during his stay in the department, with high level of procalcitonin, amenable to antibacterial treatment, however with several recurrent episodes, one of them with lung bleeding. Later CT of chest shows lung fibrosis, pneumoscintigraphy: hypo- and aperfusion zones. Bronchoscopy reveals nothing.

He’s been admitted to our clinic again this month. He had pneumonia on admission, responding to antibacterial treatment.
Diagnosis is not clear. There is a possibility that calcinosis developed after infectious disease with myocardial involvement. Bleedings indicate to some vasculitis process. Symptoms are partly similar to PXE description, but do not fit it completely. Still the genesis of the findings is not understood.

what is likely diagnosis_?
Answer Discussion :
Jose Samosc
CREST: Type V - Undifferentiated connective-tissue disease with 2 of 3 of the following scleroderma features: sclerodactyly, pitting scars, or scleroderma capillary pattern` or one of these features along with one of the following: Raynaud phenomenon, pulmonary fibrosis, or visceral involvement {esophagus, heart, kidney}` but do not meet the criteria for groups III and IV` no anticentromere antibodies` no telangiectasia
8 years ago
Samira Alfayadh
Ehler Danlos syndrome
8 years ago

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