Diagnostic accuracy of cerebrospinal fluid adenosine deaminase in detecting Tuberculous Meningitis

Objective: To determine diagnostic accuracy of Cerebro Spinal Fluid [CSF] Adenosine De-Aminase [ADA] in detecting Tuberculous Meningitis [TBM] keeping CSF Polymerase Chain Reaction [PCR] for Mycobacterium Deoxy Ribonucleic Acid [DNA] as gold standard

Methods: This cross sectional validation study was conducted at Department of General Medicine of PNS Shifa Naval Hospital Karachi, Pakistan from Oct 2015 to Mar 2017 for a total duration of one and a half year. One hundred and thirty six patients were included. The diagnosis of TBM was based clinically on symptoms like fever, headache, altered mental state and signs of meningeal irritation with CSF findings of increased proteins, low glucose and lymphocytic pleocytosis. Lumbar puncture was done and approximately 4ml of CSF sample was withdrawn for analysis. Diagnosis of TBM was confirmed by doing CSF PCR test for mycobacterium tuberculosis DNA

Results:Total 136 patients were enrolled in this study. Mean age in our study was 47.09 +/- 12.80 years, whereas frequency and percentages of male and female patients was 102 [75 percent] and 34 [25 percent] respectively. The diagnostic accuracy, sensitivity, specificity, positive predictive value and negative predictive value of CSF ADA level in detecting TBM was 71.32 percent, 84.21 percent, 95.45 percent, 98.97 percent and 53.85 percent respectively

Conclusion: The study concludes that diagnostic accuracy of CSF ADA in detecting TBM is high which is proposed as an investigation to differentiate it from other causes of meningitis in places where PCR test is not available

Vitiligo in children: a distinct subset

Objective: To determine clinical profile of vitiligo in children. Study Design: A descriptive study. Place and Duration of Study: Combined Military Hospital, Abbottabad, from January 2010 to June 2013

Methodology: All new patients below the age of 15 years, clinically diagnosed to have vitiligo, were included in the study. A detailed history was obtained, thorough physical examination was performed, and findings were recorded on a specially designed proforma for each patient separately. Computer programme SPSS-14 was used to manage and analyze the data

Results: Out of 157 children, 68 [43.3%] were males and 89 [56.7%] were females. Mean age at onset was 6.55 +/- 3.43 years. The disease started before 10 years of age in 123 [78.3%] children. Average duration of the disease was 1.48 +/- 1.87 years. Average duration of the disease was 1.73 +/- 2.09 years in male children and 1.29 +/- 1.67 years in female children. Generalized vitiligo was the commonest type [n = 83, 52.9%]. The disease started most commonly from head and neck [n = 75, 47.8%]. A family history of vitiligo was found in forty-nine [31.2%] children, Koebner phenomenon in 39 [24.8%] children and associated autoimmune or endocrine diseases in 8 [5.1%] patients

Conclusion: Majority of the children developed the disease before 10 years of age. Generalized vitiligo was the commonest type. Childhood vitiligo was more common in female children

Clinical pattern of vitiligo

All new cases of vitiligo reporting to Dermatology Outpatient of Combined Military Hospital, Panu Aqil, were included in the study. Of the 230 patients, 124 were male [53.9%] and 106 were female [46.1%]. Males were more commonly affected. The disease affected all age groups. Mean age at presentation was 27.02 +/- 18.34 years and age at presentation ranged from 5.5 months to 82 years. The mean age at onset was 22.03 +/- 16.97 years with majority 30.4% [n=70], developing vitiligo in first decade of life. Generalized vitiligo was the most common type [n=132, 57.4%] followed by focal [n=53, 23%] and aero-facial vitiligo [n=16, 7%]. Head and neck was the most common initial site of onset [n=100, 43.48%]. Koebner phenomenon was observed in 72 patietns [31.3%], family history was present in 64 patients [27.8%] and 16 patients [7%] had associated diseases

Harlequin ichthyosis in two siblings

Harlequin ichthyosis is a rare and extremely severe form of congenital ichthyosis. The affected neonates usually do not survive beyond first few days after birth, but several long-term survivals have been noted. The inheritance is thought to be autosomal recessive. It has recently been shown that the vast majority of affected individuals are homozygous for mutations in the ABCA12 gene, which cause a deficiency of the epidermal lipid transporter and result in hyperkeratosis and abnormal barrier function. Prenatal diagnosis is possible. We report a case of a newborn with Harlequin ichthyosis, a product of consanguineous marriage, with a history of similar disease leading to early neonatal death previously in a sibling

Treatment of toxic epidermal necrolysis [TEN] with low dose intravenous immunoglobulin in child

Stevens-Johnson syndrome [SJS] and toxic epidermal necrolysis [TEN, LyeII's disease] are severe, episodic, acute mucocutaneous reaction that may be caused by various factors particularly drugs. Treatment is primarily supportive care and there are no specific therapy regimens. Intravenous immunoglobulin [IVIG] has recently been shown to be a useful and safe therapy in paediatric patients with SJS/TEN, though no such case has so far been reported from Pakistan. The authors report the experience with low dose IVIG [0.1 g/kg/day for four consecutive days for treating a 3 years old boy with toxic epidermal necrolysis with favourable outcome without early complications

Efficacy and safety of oral dexamethasone pulse treatment for vitiligo

Results of previous studies on efficacy and safety of oral corticosteroid pulse treatment for vitiligo are inconsistent. The objective of this study was to assess the efficacy and safety of oral dexamethasone pulse treatment in a cohort of Pakistani patients. This is a descriptive study. Thirty patients with vitiligo were included in the study. Of these, 21 had progressive disease and 9 had stable disease. The patients were given weekly pulses of dexamethasone on 2 consecutive days every week followed by 5 days off treatment for a maximum of 24 weeks. Clinical response and side effects were evaluated at monthly intervals. Plasma cortisol levels were also monitored. After a mean treatment period of 16 + 4 weeks, progression was arrested in 18 [85.7%] of 21 patients with active vitiligo before the study. Overall, repigmentation was noted in 14 [46.6%] patients at the end of 24 weeks. The extent of repigmentation varied from less than 25% [slight] to 51% to 75% [marked]. Twenty [66.6%] patients reported one or more side effects. Plasma cortisol values were markedly decreased 24 hours after the second dose of each pulse but returned to baseline before the next dexamethasone pulse. Oral corticosteroid pulse therapy is an effective treatment modality to arrest progressive vitiligo but is only moderately effective in inducing satisfactory repigmentation. Treatment associated side effects are frequent but reversible; however, sustained suppression of endogenous cortisol production does not occur with pulse regimen