al-Fatihah buat Anas Hakimi bin Wan Umar..
i have never thought that what i've been afraid of since the day Anas was moved to ICU, eventually turn into a reality one..for all those suffering moments u had in the previous attacks your ummi told me, sometimes I cant help thinking of the worse condition u might deal with..3-4months hospitalization u used to go tru is enough to tell how much u suffer, not to mention all those interventions made up through your body..and I know how much your ummi hurts seeing u that way
never thought that when I'm coming back home this coming september I'll never meet u again.. the moment ayah cik texted me, i just couldnt believe what my naked eyes were seeing..the worst thing that I would never wish to happen, and it even sickened me for couldnt be around together with everyone for u the last few days..i hate it.
doakan cikpah nak exam..cikpah akan doa byk2 utk anas..cikpah tahu Allah sayangkan Anas thats why Dia amek Anas balik not allowing u to suffer much more..i'm gonna miss u..cikpah sayang semua :'(
The Gorham-Stout syndrome (Gorham’s massive osteolysis)
Gorham’s disease is a rare disorder characterized by proliferation of vascular channels that results in destruction and resorption of osseous matrix. Since the initial description of the disease by Gorham and colleagues (1954) and by Gorham and Stout (1955), fifty years have elapsed but still the precise etiology of Gorham’s disease remains poorly understood and largely unknown. There is no evidence of a malignant, neuropathic, or infectious component involved in the causation of this disorder. The mechanism of bone resorption is unclear.
The clinical presentation of Gorham’s disease is variable and depends on the site of involvement. It often takes many months or years before the offending lesion is correctly diagnosed. A high index of clinical suspicion is needed to arrive at an early, accurate diagnosis. Patients with Gorham’s disease may complain of dull aching pain or insidious onset of progressive weakness. In some cases, pathologic fracture often leads to its discovery. Gorham’s disease is progressive in most patients; however, in some cases, the disease process is self-limiting. The clinical course is generally protracted but rarely fatal, with eventual stabilization of the affected bone being the most common sequelae. Chylous pericardial and pleural effusions may occur due to mediastinal extension of the disease process from the involved vertebra, scapula, rib or sternum, and can be life threatening. A high morbidity and mortality is seen in patients with spinal and/or visceral involvement.
Classification of idiopathic osteolysis according to Hardegger et al
1. Hereditary multicentric osteolysis with dominant transmission - Between the age of two and seven years, spontaneous pain and swelling begin in the hands and feet. Carpotarsal osteolysis occurs over the period of a few years. Progression ceases normally in adolescence.
2. Hereditary multicentric osteolysis with recessive transmission- Similar to type 1, but may be associated with severe generalised osteoporosis.
3. Non-hereditary multicentric osteolysis with nephropathy- Appears in childhood. There is a gradual disappearance of the carpus with the tarsal bones involved, but to a less degree, and an association with proteinuria. Death occurs usually due to renal failure and malignant hypertension.
4. Gorham's massive osteolysis (Gorham-Stout syndrome) - Monocentric occurrence in any part of the skeleton may start at any age. Normally ‘haemangiomatous tissue’ is found in the osteolytic region. It has neither a hereditary pattern nor an associated nephropathy. The disease is benign and the osteolysis usually stops after a few years.
5. Winchester syndrome Autosomal recessive transmission- Rare childhood carpotarsal osteolysis inassociation with contractures, shortness of stature, skin lesions, corneal clouding and osteoporosis without nephropathy.