Qatar Medical Journal - Volume 1998, Issue 2

Omenn syndrome is a rare form of severe combined immune deficiency (SCID) with distinctive clinical and immunologic features⁽¹⁾. It was first described by Omenn in 1965 under the title of: Familial Reticuloen-Dotheliosis

with Eosinophilia^(2),. Since then, over 45 cases has been described⁽¹⁾. In this paper; we are reporting the first case of Omenn's syndrome in Qatar community.

Sarcoidosis is an idiopathic granulomatous disease involving one or more multiple organ systems, characterised by the histologic finding of noncaseating epitheloid cell granulomas. This is the first report of familial sarcoidosis in Jordan. We report a mother and her son who Presented with bilateral hilar lymphadenopathy and lung infiltrates.

Sarcoidosis is a rare disease in Jordan. We reported thirty-three cases of sarcoidosis⁽¹⁾. Although the familial tendency is reported in many studies⁽²⁾, this was not the case in our study. Though the etiology is unknown; both Prevalence of sarcoidosis in different ethnic groups and familial occurrence of the disease strongly suggest the hypothesis of a possible genetic predisposition⁽³⁾.

A 32 year old lady reported recurrent attacks of chest tightness and dyspnoea since childhood and a recent history of intermittent attacks of haemoptysis. A chest X-ray prompted further investigations which revealed isolated unilateral pulmonary artery agenesis (UPAA). She underwent right pneumonectomy and had a smooth post-operative recovery. She has been followed for 12 months and is enjoying good health.

A patient with sexually transmitted human immuno-deficiency virus and hepatitis B infections is described. Diagnosis of human immunodeficiency virus infection was made incidentaly when he donated blood. He subsequently developed a self-limiting acute hepatitis B infection. The case is presented and the risks of viral transmission from blood transfusion during the period before seroconversion are discussed.

A report of a 55-year-old white Jordanian female, who presented in March 1990 with a dry cough and progressive shortness of breath of two years duration.

The chest X-ray showed bilateral, multliple and widespread nodular lesions which was confirmed by a chest CT scan.

Diagnosis was proved by an open lung biopsy and special immunohistochemical staining for factor VIII to be Epilthelioid Hemangioendothelioma of the lung. (Intravascular, bronchiolar and alveolar tumor).

We think that this is the first case that has dust exposure? and has been reported in Jordan.

Rupture of a normal spleen during pregnancy is very rare. We report a case of splenic rupture following an unclear history of falling in third trimester of pregnancy. Abruptio placenta was suspected preoperatively but, after caesarean section, exploration of the abdominal cavity revealed a ruptured spleen and splenectomy was performed. Disseminated intravascular coagulopathy (DIC), renal failure, adult respiratory distress, necrotizing enterocolitis and bilateral hydrothorax were complications which were managed successfully.

A case of rhino-cerebral mucormycosis is described which is the first such case report from Qatar; The patient was a young female with systemic lupus erythematosis and was on regular dialysis for renal failure. She was admitted primarily because of diarrhea but her condition was complicated by progressive swelling of the face and eyes, total ophthalmoplegia, cavernous sinus thrombosis, a lower motor neurone facial nerve lesion and contralateral hemiplegia. Despite aggressive supportive efforts and Amphotericin-B therapy, she died. Although the disease is a potentially fatal fungal infection, cure can be achieved if the case is properly managed, hence the value of reporting the case.