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September 2009 KUWAIT MEDICAL JOURNAL 253 and adrenal gland [1] . The adverse effects of CS are prominent due to the high dose required (40 to 80 mg prednisolone) [8] ; pulse therapy with methyl prednisolone maybe used in certain cases. Scott et al [9] recently advocated early intervention with immunosuppressive agents in patients who present with disabling symptoms with favorable outcomes and minimal treatment related toxic effects. Hydroxychloroquine has been used for hypercalcemia, skin and neurological involvement [2] . Chlorambucil, cyclophosphamide, cyclosporine and azathioprin have all been used as steroid sparing agents with variable degrees of success. Radiation therapy also has been tried in refractory NS. A recent article described a case of steroid refractory NS with marked clinical and radiological response to infliximab; a chimeric monoclonal human-murine antibody directed against tumor necrosis factor alpha [10] . Neurosurgical intervention is indicated for selected cases such as ventriculoperitoneal shunting for obstructive hydrocephalus or resection of intracranial granulomas causing raised intracranial pressure [8] . In our case, in spite of the dramatic presentation that was suggestive of meningo-encephalitis due to the high grade fever, leucocytosis and diminished sensorium, an urgent brain CT scan insinuated a plethora of differential diagnoses and a simple CXR limited them to only a few. However, the definitive diagnosis was offered by the histopathological examination of a cervical lymph node coupled by brain MRI findings and confirmed by the good response to steroids. REFERENCES 1. Burns TM. Neurosarcoidosis. Arch Neurol 2003; 60:1166-1168. 2. Iannuzzi MC, Rybicki BA, Teirstein AS. Sarcoidosis. N Engl J Med 2007; 357:2153-2165. 3. Uchino M, Nagao T, Harada N, Shibata I, Hamatani S, Mutou H. Neurosarcoidosis without systemic sarcoidosis - case report. Neurol Med Chir (Tokyo) 2001; 41:48-51. 4. Spencer TS, Campellone JV, Maldonado I, Huang N, Usmani Q, Reginato AJ. Clinical and magnetic resonance imaging manifestations of neurosarcoidosis. Semin Arthritis Rheum 2005; 34:649-661. 5. Sponsler JL, Werz MA, Maciunas R, Cohen M. Neurosarcoidosis presenting with simple partial seizures and solitary enhancing mass: case reports and review of the literature. Epilepsy Behav 2005; 6:623- 630. 6. Kellinghaus C, Schilling M, Ludemann P. Neurosarcoidosis: clinical experience and diagnostic pitfalls. Eur Neurol 2004; 51:84-88. 7. Nunes H, Brillet PY, Valeyre D, Brauner MW, Wells AU. Imaging in sarcoidosis. Semin Respir Crit Care Med 2007; 28:102-120. 8. Joseph FG, Scolding NJ. Sarcoidosis of the nervous system. Pract Neurol 2007; 7:234-244. 9. Scott TF, Yandora K, Valeri A, Chieffe C, Schramke C. Aggressive therapy for neurosarcoidosis: long-term follow-up of 48 treated patients. Arch Neurol 2007; 64:691-696. 10. Toth C, Martin L, Morrish W, Coutts S, Parney I. Dramatic MRI improvement with refractory neurosarcoidosis treated with infliximab. Acta Neurol Scand 2007; 116:259-262.
254 KUWAIT MEDICAL JOURNAL September 2009 Case Report Urosepsis Simulating Congenital Adrenal Hyperplasia in an Infant Fahad Al-Mukhaizeem 1 , Abdullah Shamsah 2 , Homoud Taher 3 1 Department of Pediatric Emergency Medicine, Mubarak Al-Kabeer Hospital, Kuwait 2 Department of Pediatric Emergency Medicine, New Dar Al-Shifa Hospital, Kuwait 3 Department of Family Medicine, Mishrif Polyclinic, Kuwait Kuwait Medical Journal 2009; 41 (3): 254-256 ABSTRACT Urinary tract infection in infants simulating congenital adrenal hyperplasia is rarely reported in literature. We report a case, which was misdiagnosed as a case of congenital adrenal hyperplasia. A three-month old female infant was admitted to a local hospital for persistent vomiting and severe dehydration. Her biochemical profile revealed hyponatremia, hyperkalemia and metabolic acidosis. She was initially misdiagnosed as congenital adrenal hyperplasia (CAH). Her urine examination revealed a urinary tract infection. Upon investigation there was no evidence of an obstructive uropathy. A full septic work up was performed and she was treated for a suspected urosepsis. Her electrolytes imbalance improved to normal and she had a complete recovery after antibiotic therapy. This case demonstrates the importance of urine culture and ultrasound examination in suspected case of pseudo-hypoaldosteronism KEY WORDS: congenital adrenal hyperplasia , pseudo-hypoaldosteronism , transient pseudohypoaldesteronism , urosepsis INTRODUCTION Pseudo-hypoaldosteronism (PHA) was described as a salt losing condition seen in infants with vomiting, dehydration, hyponatremia, hyperkalemia, metabolic acidosis, and increased plasma aldosterone concentration with normal adrenal function and normal renal anatomy [1] . In addition to sporadic cases, familial, recessive and dominant inheritance has been described [2] . PHA is a rare salt wasting syndrome of infancy and is postulated to be caused by renal tubular insensitivity to mineralocorticoids. It may be an isolated condition or may be associated with renal disease [3] . A wide variety of urologic lesions have been reported in association with transient pseudo-hypoaldosteronism (TPHA), these include obstructive and non-obstructive lesions [4] . The prompt diagnosis of the potentially fatal congenital adrenal hyperplasia (CAH) in neonate is critical. However, neonatal infection is actually more common. Early onset neonatal infection occurs within the first five days of life. Infection is caused by organisms acquired during intra-uterine or intra-partum stages. Late onset neonatal infection occurs after seven days and frequently results from colonization. Escherichia coli is one of the most important pathogen of new born and young infants causing sepsis, meningitis, and urinary tract infection [5] . We report an unusual case of neonatal urosepsis secondary to E.coli, with clinical presentation simulating CAH. We will review the literature with emphasis on the differential diagnosis and treatment of this condition. The purpose of this communication is to report the apparent association of urinary tract infection in a female neonate who had multiple electrolytes abnormalities and the adrenogenital syndrome. CASE HISTORY A three-month-old girl born at term by spontaneous vaginal delivery was seen in a community hospital with a one week history of persistent vomiting, increased irritability and decrease feeding. She had previously been fit and well. On the day of admission the parents reported that the baby was lethargic and difficult to arouse. On assessment at the community hospital, the infant was found to be severely dehydrated. Physical examination revealed a heart rate of 190 beats/min, a respiratory rate of 48 breaths/min, capillary refill of five seconds, a blood pressure of 84/54 mmHg and a temperature of 37.2 o C. Chest and abdominal examinations were unremarkable, and the infant had normal external genitalia with no clitromegaly and no hyperpigmentation. She was initially treated with a 20 ml/kg bolus of intravenous saline (0.9 % Address correspondence to: Dr. Fahad Al-Mukhaizeem, MD, FRCPC, FAAP, Department of Pediatric Emergency Medicine, Mubarak Al-Kabeer Hospital, Kuwait.
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Vol 41 # 3 September 2009 - Kma.org.kw

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