A case of orofacial granulomatosis evolving into Melkersson Rosenthal syndrome in a child treated with a steroid free regimen of Clofazimine and Minocycline.

Orofacial granulomatosis (OFG) is a rare disorder with varied etiological, immunological and infectious mechanisms implicated and is believed to be a umbrella term which includes Melkersson Rosethal syndrome (MRS). We describe a 17 year old female who was diagnosed with OFG and was successfully treated with a combination of minocycline and clofazimine without oral steroids with significant improvement within 1 month of therapy.

Severe type 2 leprosy reaction with COVID-19 with a favourable outcome despite continued use of corticosteroids and methotrexate and a hypothesis on the possible immunological consequences.

Type 2 leprosy reaction (T2LR), or Erythema Nodosum Leprosum (ENL), often poses a therapeutic challenge to clinicians and commonly requires long courses of steroids for control. While immunosuppressants are known to achieve control and lower steroid dependence in T2LR, the prospect of managing a severe T2LR in conjunction with COVID-19, with the concern of worsening COVID-19 with long-term immunosuppression has not previously been encountered. We report a case of severe T2LR treated with oral steroids and methotrexate, with COVID-19 infection acquired during hospital stay, and a favourable outcome achieved despite the continued use of immunosuppressants. We discuss the possible reasons for this both in terms of the drug pharmacodynamics and the immunological profile of T2LR.

Treating perinatal asphyxia with theophylline at birth helps to reduce the severity of renal dysfunction in term neonates.

AIM: Perinatal asphyxia is a common neonatal problem and contributes significantly to neonatal morbidity and mortality. This study was designed to determine whether theophylline could prevent or ameliorate renal dysfunction in term neonates with perinatal asphyxia. METHODS: We randomised 159 severely asphyxiated term newborns to receive a single dose of 5 mg/kg intravenous theophylline (n = 78) or a placebo (n = 81) during the first hour of life. The infant's 24-hour fluid intake, urine volume, serum creatinine, creatinine clearance and sodium excretion were recorded during days one, three and five of life, starting 12 hours after the theophylline or placebo infusion. RESULTS: Neonates in the theophylline group had lower serum creatinine levels (0.83 ± 0.35 versus 1.47 ± 0.61; p = 0.00) and higher endogenous creatinine clearance (32.16 ± 16.34 versus 17.73 ± 7.92; p = 0.00) than the placebo group. Severe renal dysfunction, namely acute kidney injury, was present in 36 (15%) of the neonates in the theophylline group versus 117 (48%) in the placebo group (p < 0.01). CONCLUSION: A single dose of intravenous theophylline administered to term neonates with perinatal asphyxia within the first hour of life significantly decreased serum creatinine levels and significantly increased creatinine clearance.

Molecular cytogenetics in childhood acute lymphoblastic leukemia: a hospital-based observational study.

OBJECTIVE: This study was conducted to determine the frequency of chromosomal aberrations in children aged <19 years with newly diagnosed acute lymphoblastic leukemia (ALL), attending/admitted in the Department of Pediatrics and Radiotherapy, Government Medical College, Jammu. Furthermore, we aimed to study the correlation between the cytogenetic molecular abnormalities and the immediate clinical outcome (induction of remission). MATERIALS AND METHODS: This was a prospective study conducted over a period of 2 years (May 2011 to May 2013) in a tertiary care hospital in India. Forty pediatric (1-19 years) patients (18 males, 22 females; M: F = 0.8 : 1) with newly diagnosed ALL were studied for molecular cytogenetic analysis. Written consent was obtained from the parents of the patients. Bone marrow aspiration was done for making the diagnosis of ALL. Children lost to follow-up and who failed to give consent were excluded from the survey. Host factors and clinical parameters were obtained from patients. RESULTS: Bone marrow aspirate samples of 40 diagnosed cases of ALL were subjected to routine cytogenetic analysis, and reverse transcription-polymerase chain reaction (RT-PCR) technique was used for molecular analysis. Well-spread metaphase plates were obtained in 18/40 (45%) cases for analysis. RT-PCR revealed abnormal genes in 20/40 (50%) patients. The results of molecular cytogenetic analysis were correlated with patients' clinical and hematological parameters for risk stratification and immediate outcome (induction of remission). Eighteen out of 40 (45%) cases revealed no abnormality. Among the remaining 22 cases, 8 had TEL-AML1 (20%), 6 had BCR-ABL (15%), 4 had MLL-AF4 (10%), 2 had E2A-PBX1 (5%) fusion genes, and 2 had hyperdiploidy. To conclude, a higher proportion of cases in this study showed adverse translocations such as t (9;22), t (4;11), and t (1;19) compared to that reported in literature. CONCLUSION: RT-PCR assay was useful in detecting the prognostically significant oncogene fusion transcripts. In our study of 40 patients, we found that the pattern and frequency differ from those reported in Western literature. Our study reveals a lower frequency of hyperdiploidy (5%) and a higher frequency of BCR-ABL gene fusion (20%) in childhood ALL. Above all, in contrast to previous studies on childhood ALL, our study showed female predominance, with the male-to-female ratio being 0.8 : 1. Apart from the BCR-ABL fusion gene, none other was associated with poor prognosis. It is already well established that the characterization of the genetic entities at diagnosis is crucial for the understanding and the optimal treatment of ALL. Because the aberrations in our population differ significantly from those reported in Western populations, we may be required to tailor our protocols.

Occurrence of plant sensitivity among patients of photodermatoses: a control-matched study of 156 cases from New Delhi.

BACKGROUND: Photodermatitis is an abnormal response to ultraviolet radiation (UVR). The photoallergic contact dermatitis caused by plant allergens is a serious cause of morbidity in India. Airborne contact dermatitis is the classical presentation of plant-induced dermatosis, which may become difficult to differentiate from chronic actinic dermatitis in chronic cases. The rapid growth of parthenium weed in India and its ill effects on the population make it important to detect all cases of parthenium sensitivity, which in some cases might simulate photodermatitis. AIMS: This study aims to detect the occurrence of plant sensitivity and photosensitivity in idiopathic-acquired photodermatoses, airborne contact dermatitis and general population taken as controls. METHODS: One hundred and fifty six consecutive patients suffering from polymorphic light eruption (PMLE), chronic actinic dermatitis (CAD) and airborne contact dermatitis (ABCD) were enrolled in the study over a period of three years (June 2004 to May 2007). An equal number of age and sex matched healthy subjects were enrolled in the study as controls. All the patients were subjected to detailed history taking, clinical examination and histopathological examination for diagnosis. Patch and photopatch testing were perfomed in all the patients and healthy controls for detection of allergic and photoallergic reactions to parthenium, xanthium and chrysanthemum plant antigens and control antigens. RESULTS: Out of 156 patients enrolled in the study, 78 (50%) had CAD, 67 (42.9%) had PMLE and 11 (7.05%) had ABCD. The occurrence of parthenium/xanthium allergy and photoallergy, either to parthenium or both was most commonly found in ABCD (72.7%), followed by CAD (32%). In PMLE 4.5% cases showed photoallergy. Only 1.9% in the control group showed sensitivity to parthenium and xanthium. CONCLUSION: This study indicates that parthenium (and possibly xanthium) may act as important environmental factors in the initiation and perpetuation of not only ABCD but of CAD as well. Photoexacerbation to UVA at positive parthenium/xanthium sensitivity sites in ABCD and CAD indicates that ABCD with photosensitivity to compositae can lead to CAD.