Extrameningeal complications of meningococcal septicaemia occur in about 11–19% of cases, and include myocarditis, acute renal failure, arthritis, pneumonia, skin gangrene, conjunctivitis, endocarditis, pericarditis, endophthalmitis, urethritis, WaterhouseFriderichsen syndrome, vasculitis, and digital ischaemia. We describe the use of a prostacyclin analogue in the treatment of cutaneous digita...

The compression of the proximal part of the celiac trunk by median arcuate ligament of the diaphragm during expiration is defined as median arcuate ligament syndrome. It is a rare cause of chronic mesenteric ischemia. We report two cases with this syndrome, primarily diagnosed by Doppler ultrasound. The diagnosis was confirmed with digital substraction and computed tomography angiography in bot...

Juberg R C, Hayward J R. A new familial syndrome of oral, cranial, and digital anomalies. J Pediatr 1969; 74: 755-62. 2 Nevin N C, Henry P, Thomas P T S. A case of the orocraniodigital (Juberg-Hayward) syndrome. J Med Genet 1981; 18: 478-80. 3 Savage D C L, Swift P G F, Johnston P G B, Goldie D J, Murphy D. Combined test of anterior pituitary function in children. Arch Dis Child 1978; 53: 301-4...

The clinical manifestations of 88 children with congenital constriction band syndrome involvement of the hand were reviewed. Seventy-five of these children had evidence of digital or limb amputations, with 235 upper limb amputations and 138 lower limb amputations. In the hand, digital amputations were most common in the index, middle, and ring fingers, whereas in the foot, amputations of the ha...

Moyamoya syndrome has rarely been reported in association with Down syndrome. We report a case of an 11-year-old female child with Down syndrome who presented with hemiparesis and facial palsy. Imaging investigations (magnetic resonance angiography and digital subtraction angiography) revealed the classical Moyamoya pattern. Work-up for prothrombotic and autoimmune disorders was negative. The n...

Presented here is a case of a 8 year old boy with typical clinical manifestations of Acrocallosal syndrome. The characteristic features of this syndrome are craniofacial abnormalities, distinctive digital malformation, mental retardation. The clinical and major nosologic aspects of this condition are discussed.

Six children with Aarskoga's facial-digital-genital syndrome are described, and the genetics of the condition discussed. We suggest that this anomaly may be fairly common and that a syndrome identification centre could lead to earlier diagnosis of children with this and other syndromes.

A 75-year-old right-handed woman presented to the emergency department with simultanagnosia and right unilateral optic ataxia. Moreover, the patient had agraphia, acalculia, digital agnosia and right-left disorientation, consistent with complete Gerstmann's syndrome. This case highlights the concurrence of Gerstmann's syndrome and unilateral optic ataxia in the acute phase of a left middle cere...

A mother and son are reported with chronic dacrocystitis, cup shaped ears, hearing loss, abnormal teeth, and poor formation of saliva and tears. They are similar to previously reported cases of lacrimo-auriculo-dento-digital (LADD) syndrome. The variability of expression of this autosomal dominant syndrome is discussed, and it is suggested that poor saliva and tear formation be added to the phe...