Association of peripheral multifocal choroiditis with sarcoidosis: a study of thirty-seven patients

OBJECTIVE: To assess the clinical spectrum of peripheral multifocal choroiditis (PMC) and its association with sarcoidosis. METHODS: Thirty-seven patients examined between November 1997 and November 2001 who met all diagnostic criteria for PMC were included in this retrospective study. Patients were assessed for the following signs of sarcoidosis: typical changes on chest radiography or computed tomography; predominantly CD4 lymphocytosis in bronchoalveolar lavage fluid; elevated serum angiotensin-converting enzyme levels; elevated gallium uptake; and noncaseating granuloma on biopsy. RESULTS: Most of the patients were female (30 of 37; 81%) and white (30 of 37; 81%). Mean +/- SD age at onset was 57.5 +/- 18.7 years. Seven (19%) of the 37 patients had biopsy-proven sarcoidosis and 18 patients (49%) with presumed sarcoidosis met at least 2 of the above-mentioned criteria for sarcoidosis but had normal biopsy results. Twelve patients (32%) had an indeterminate diagnosis. Patients with presumed sarcoidosis did not differ from those with proven sarcoidosis as regards the above-mentioned criteria, except for noncaseating granuloma, implying that more than two-thirds of patients (predominantly whites) had underlying sarcoidosis. Most patients with positive gallium scintigraphy had increased mediastinal uptake, as described in sarcoidosis. Patients with underlying sarcoidosis had more severe visual impairment due to cystoid macular edema (CME). Weekly methotrexate (0.3 mg/kg) seemed to control CME. CONCLUSION: White patients with PMC should be considered to have sarcoidosis. The identification of sarcoidosis in patients with severe ocular disease can help with therapeutic choices.     

Adverse effects of pesticides on biochemical and haematological parameters in Tunisian agricultural workers

Biomonitoring of effects in agricultural workers is necessary to assess the individual risk of handling pesticides. In this study, biochemical and haematological parameters were measured to evaluate the effects of exposure to these compounds in agricultural workers. The study was carried out in 110 workers and 97 control subjects. Several haematological and biochemical parameters were analysed. Assessment of haematological parameters revealed that the mean cell volume and haematocrit levels were significantly lower in workers than in controls (P=0.002 and 0.013, respectively), while mean corpuscular haemoglobin concentrations were higher in workers (P<0.001). There was also a significant inhibition of butyrylcholinesterase activity in workers compared with that in controls (P<0.001). Assessment of biochemical parameters further showed significantly higher activities of transferases, lactate dehydrogenase (P<0.001), alkaline phosphatase (ALP) (P=0.006) and creatine kinase (CK) (P<0.015), as well as higher levels of proteins (P<0.001), creatinine (P=0.001) and urea (P=0.001) in workers compared with controls, along with significantly higher uric acid levels (P=0.012). Furthermore, the number of years exposed to pesticides predicted higher activities of alanine aminotransferase, CK, ALP, as well as uric acid levels. Overall, chronic exposure to pesticides appeared to affect several biochemical parameters. These biomarkers seem to be indicative of adverse effects of pesticides in agricultural workers, confirming their use for routine monitoring of effects.     

Cherubism: a rare case report with literature review

Cherubism is an autosomal dominant disorder caused by a mutation of the gene encoding the binding protein SH3BP2. However, non-hereditary forms are observed, probably related to a de novo mutation. It is clinically manifested by an enlargement or a deformation of the jaw associated with a malposition of the teeth. In severe forms, these deformations can have a psychological impact, associated with ocular complications, as well as a reduction of the pharynx lumen and phonation disorders. The appearance of the lesions on imaging is not very specific, however it remains essential for the mapping of the lesions. We report a case of cherubism in a 12-year-old boy with no family history. This case report''s objective is to identify the radiological aspect of this pathology and establish an early diagnosis to oriented therapeutic interventions.     

Symptomatic congenital hemangiomatosis in a neonate: Imaging of a life-threatening presentation with multifocal liver involvement

Hemangiomas are the most common benign vascular neoplasms of infancy. Congenital hemangiomas proliferate in utero, and are fully formed at birth. They are usually solitary. Generalized forms are exceptional. The liver is the second most common site of hemangiomas after the skin. When >5 cutaneous hemangiomas are present, screening abdominal ultrasound is recommended. Based on the degree of liver parenchyma involvement, 3 hepatic hemangiomas’ subtypes are defined: focal, multifocal, and diffuse. Hepatic hemangiomas’ clinical presentation varies from asymptomatic to life-threatening. High output cardiac failure, consumptive coagulopathy, abdominal compartment syndrome, and liver dysfunction are possible complications. We report an unusual case of symptomatic congenital hemangiomatosis in a male infant born with innumerable generalized cutaneous hemangiomas whose screening abdominal ultrasound revealed multifocal hepatic hemangiomas with extensive mixed shunts. We aim to highlight this unique entity with severe associated complications and stress the role of imaging at initial presentation, for follow-up, and to guide therapeutic choices.     

Phace Syndrome in Children: Two Case Reports

PHACE(S) syndrome combines: posterior fossa brain malformations, face hemangioma, arterial cerebrovascular abnormalities, cardiovascular abnormalities, eye abnormalities, and ventral developmental defects (Sternal defects or supra-umbilical rope). The diagnosis is based on the association of an infant hemangioma exceeding 5cm in size on the face, neck, scalp with 1 major criteria or 2 minor criteria. Imaging, especially Gadolinium MRI and MRA of the brain, neck, and aortic arch, transthoracic echocardiography, or even cardiac MRI play a key role in the detection of associated neurological and cardiovascular abnormalities.We report 2 cases of PHACE syndrome revealed by 2 different clinical presentations.     

Severe hypercalcemia complicated by acute pancreatitis revealing generalized bone lysis metastasis: Case report and review

Malignant hypercalcemia is a frequent metabolic complication of osteophilic tumors, exceptionally revealing cavitary cancer, but its prognosis remains poor despite early and adequate management. We report the case of a young patient, smoker without any previous history, admitted for the management of a digestive symptoms made of abdominal pain with food vomiting. The patient had an electrocardiogram withchest computed tomography scan and BBC evoking PA on malignant hypercalcemia. An etiological investigation was conducted to confirm the tumoral origin of the hypercalcemia. We put the patient on hyperhydration with corticotherapie and biohosphonates with a good clinical and biological improvement. Malignant hypercalcemia affects about 10%-20% of patients with cancer including nasopharyngeal carcinoma. Its clinical presentation varies according to the extent and speed of onset, responsible for multivisceral involvement including kidney, heart, neuropsychiatric system, which may engage the patient''s vital prognosis. The therapeutic management is based on 4 main principles; hyperhydration, increase of urinary calcium excretion by loop diuretics, decrease of bone resorption by biphosphonates and extrarenal purification which remains the ideal choice in case of life-threatening severe hypercalcemia. Acute hypercalcemic pancreatitis as a mode of revelation of cavum cancer has almost never been described in the literature