Tuberculosis control in India: Refocus on nutrition.

Many western societies have eliminated tuberculosis years before the advent of potent anti-tuberculous drugs, as a result of the improved standards of living and good nutrition. But even with the availability of powerful anti-tuberculous drugs, India still has a long road ahead to reach the "End TB by 2025" goal. One of the major reason is that tuberculosis control program in India till now have focused primarily on case detection and medical treatment of active tuberculosis. Drug treatment alone does not completely prevent the occurrence of new infections in the community and also contributes to development of drug resistant strains if used improperly or incompletely. Although the treatment of active cases can reduce the period of transmission of disease, a significant amount of transmission to contacts occurs even before they have been diagnosed and treated. Additionally, this approach cannot prevent re-activation to active TB in the vast pool of persons with latent TB infection. Tuberculosis occurs in those with suppressed cell mediated immunity mainly due to poor nutritional status. Improving the nutritional status of the society by several social interventions hand-in-hand with utilizing the available anti-tuberculous drugs is possibly the only effective strategy. Promising programmatic guidance for nutritional support in TB patients have been formulated by the Central TB division of India but it needs a refocusing of TB control strategies towards nutrition at all levels and strong public health actions for effective implementation.

Kozhikode criteria for diagnosing systemic lupus erythematosus as a hematological disorder.

Systemic lupus erythematosus (SLE) is an autoimmune disease in which cells and tissues undergo damage mediated by tissue-binding autoantibodies. At its onset, it may involve one organ alone or more than one organ simultaneously; over a time, additional manifestations due to the involvement of other organs may occur. Our observations have confirmed that hematological manifestations are the commonest initial presentation in SLE. The criteria used till 2012 was the American College of Rheumatology (ACR) criteria, which is only a classification criterion and not really for diagnosis; if we rely on ACR criteria, the diagnosis is often delayed. Time required for satisfying all four of the eleven criteria is variable and prolonged. Moreover, hematological manifestations are underrepresented in the ACR criteria. Based on the clinical observations made on patients evaluated in a tertiary center in North Kerala, an alternate diagnostic criterion named the Kozhikode criteria was proposed, especially for the diagnosis of SLE when it presents with hematological manifestations alone. The present study was an attempt to validate the same and to look for any association of diet and lifestyle with the disease.

Cowden syndrome with Lhermitte- Duclos disease presenting as ataxia.

Cowden syndrome or multiple hamartoma syndrome is a rare genodermatosis of autosomal dominant inheritance characterized by multiple hamartomatous lesions of ectodermal, mesodermal and endodermal origin. A 45-year-old man presented to us with a history of dural arteriovenous fistula and intracerebral bleed in the past with gradually progressive difficulty in walking. Magnetic resonance imaging (MRI) of the brain showed a heterogeneous lesion in the cerebellum which was diagnosed as adult Lhermitte-Duclos disease which is considered a component of Cowden syndrome. On examination we found florid skin and mucosal manifestations of Cowden syndrome. A family history of thyroid malignancy was also present. Using the Cleveland Clinic web calculator, the patient had an 82% chance of having a phosphatase and tensin homologue (PTEN) mutation.

Unusual Case of ALL with Ovarian Relapse.

Patients with acute lymphoblastic leukemia (ALL) can develop relapse in sanctuary sites like brain, ovary or testis even when the bone marrow is in remission. Pelvic recurrence is rarely reported during the follow up of successfully treated ALL in females. We report here a very unusual case of a large pelvic lump which the patient herself could feel, that was probably an ovarian relapse of ALL, successfully treated with re-induction chemotherapy alone and achieved complete remission.

Hypertension as a Risk Factor for Haemorrhagic Stroke in Females.

AIM: To study the effect of hypertension as a risk factor for haemorrhagic stroke in women compared to men. METHOD: Confirmed cases of haemorrhagic stroke by Computed Tomography scan were included in this study. The participants underwent detailed clinical examination based on preset questionnaire. Relevant laboratory investigations were done in this group. 100 consecutive cases were studied comparing the baseline characteristics with male patients admitted with haemorrhagic stroke. OBSERVATIONS: Among all the risk factors studied hypertension was the single most important risk factor causing intracerebral bleed. Forty seven point four percentage (47.4%) of females were hypertensive as against 61% in males. Blood pressure recording of females showed lower systolic and diastolic measurement compared to males and the difference was statistically significant with a p value of 0.000. Majority of the female patients were overweight with a significant difference in body mass index compared to males. (p value 0.006). CONCLUSION: Hypertension is an important risk factor for intracerebral bleed. Females developed intracerebral bleed at a lower blood pressure measurement compared to males. Females require better control of hypertension than males to prevent intracerebral bleed.

Emphysematous pyelonephritis in a patient infected with the human immunodeficiency virus.

Emphysematous pyelonephritis (EPN) is a rapidly progressive and life-threatening infection that is seen most commonly in persons with diabetes. The infecting organisms usually consist of mixed flora, including Escherichia coli (68%), Klebsiella pneumoniae (9%) and Proteus mirabilis. Females are affected twice as often as men, and mortality rates can be as high as 80%. Obstructive uropathy, urinary calculi, calyceal stenosis and neoplasms are significant predisposing factors. We report a case of EPN in a patient with the human immunodeficiency virus infection, without diabetes mellitus or urinary tract obstruction, which responded remarkably to conservative management with antibiotics alone.

Hematological Manifestations of SLE at Initial Presentation: Is It Underestimated?

SLE can present with hematological manifestations alone or along with features of other system involvement. With a low index of clinical suspicion or inadequate follow up the diagnosis may be delayed or missed at the time of presentation, in those with hematological abnormalities as the initial manifestation. An observational study was conducted among patients of SLE, in a tertiary referral centre of North Kerala, with the purpose of estimating the proportion of patients with hematological manifestations as the initial presentation of the disease and to study their nature. It was observed that 82% of the patients had hematological manifestations at presentation. It is the most common presenting manifestation of SLE in people of North Kerala. Autoimmune hypothyroidism was one of the common coexisting abnormalities in these patients, which is not included in the American College of Rheumatology (ACR) criteria for diagnosis. Arthritis was uncommon among those who presented with hematological manifestations. A significant number of patients do not satisfy the ACR criteria at the time of diagnosis but do so on follow up. The ACR criteria are weak to diagnose such patients and therefore need revision. We therefore propose an alternative to ACR criteria as "Kozhikode criteria for SLE".

Cerebral vein thrombosis misdiagnosed and mismanaged.

Cerebral venous thrombosis (CVT) should be considered in the differential diagnosis of all unexplained CNS disorders of sudden onset. Etiological factors are often subclinical forms of several common thrombophilic states occurring together, rather than the typical inherited and rare causes. Diagnosis is missed because of the heterogeneity in clinical presentation and etiological factors. In several patients with the so called idiopathic CVT nutritional deficiencies and lifestyle issues are more important factors in pathogenesis, rather than single rarer causes. High index of suspicion is the key to diagnosis. Clinical skill has to be fine tuned to diagnose the problem and to identify all the etiological factors. Radiology is essential for diagnosis but relying on radiology alone will lead to missing several cases and even erroneous diagnosis. It is inappropriate to proceed prematurely to laboratory investigations, forgetting proper clinical evaluation by studying diet, lifestyle, and environment of the patients. Success in managing lies in identifying all the contributory causes and correcting all of them giving excellent outcome almost always. Clinical observations based on case series and sharing of such information alone are the means to arrive at a consensus in diagnosis and management.

AIDS associated Kaposi's sarcoma.

Epidemic Kaposi's sarcoma (KS) is the most common malignant neoplasm occuring in HIV patients. Although the prevalence of HIV infection is high in Asian countries, KS is rarely reported. We report a case of fatal KS involving the skin and mucous membranes in a homosexual male.

Primary haemophagocytic syndrome in a young girl.

Haemophagocytic syndrome is a life-threatening systemic illness characterized by an uncontrolled inflammatory response. Patients present with fever, hepatosplenomegaly, jaundice and liver dysfunction, neurological manifestations and often pancytopenla. Bone marrow, lymph node, hepatic or splenic biopsy shows macrophages with Ingested blood cells or their precursors. Laboratory markers include elevated triglycerides and ferritin, low fibrinogen with normal or low erythrocyte sedimentation rate (ESR). Familial haemophagocytic lymphohistiocytosis (HLH) is an autosomal recessive disorder. Secondary haemophagocytic syndrome results from infections, malignancy and collagen vascular disorders. We describe a young girl with primary haemophagocytic syndrome.

Cortical venous thrombosis due to acquired hyperhomocysteinaemia.

A 20-year-old student presented with generalized tonic-clonic seizures and was diagnosed to have cortical venous thrombosis. Her dietary history and the clinical signs of vitamin deficiency prompted further investigations, which detected hyperhomocysteinaemia secondary to vitamin B12 deficiency as a factor contributing to the hypercoagulable state. This case highlights the importance of a balanced diet, as well as the necessity for primordial prevention.

Congenital immunodeficiency disorder.

A 17 year old girl, a 9th standard student with history of recurrent pneumonia and soft tissue "cold abscesses" since neonatal period, presented with fever and cough with yellowish expectoration of 2 months duration. Her clinical and radiological finding along with elevated serum IgE level were consistent with the diagnosis of hyper immunoglobulin E syndrome or Job's syndrome.

Idiopathic hypoparathyroidism presenting as secondary myelofibrosis.

Myelofibrosis occurring both in childhood and adulthood is most commonly idiopathic, followed by neoplastic infiltration of the bone marrow. We are reporting an extremely rare association of idiopathic hypoparathyroidism associated with stable secondary myelofibrosis.

PUO due to Langerhans Cell Histiocytosis.

Langerhan Cell Histiocytosis (LCH) is a disorder in which cells with a phenotype similar to that of epidermal langerhans cells cause tissue damage possibly through excessive cytokine production. The clinical spectrum of the disease is wide. We are reporting a case of LCH who presented with prolonged pyrexia and a clinically benign bony swelling of mandible of long duration, which was otherwise ignored as being unrelated. The biopsy from the swelling confirmed the diagnosis of LCH. Another biopsy from lower end of tibia where he had pain also demonstrated typical findings. There was no evidence of other system involvement. There was good remission of the swellings and the symptoms with steroid alone initially but later relapsed and is now on treatment as per LCH III protocol. The case is being reported for its rarity and for the unusual presentation as PUO.

Restrictive cardiomyopathy due to primary plasma cell leukemia.

The causes of restrictive cardiomyopathy are numerous, of which neoplastic infiltration is also known. Plasma cell leukemia is considered as the, most severe form of multiple myeloma, is an extremely rare condition. Among them, primary plasma cell leukemia has got an incidence of one in one million only. We report a case summary of a patient who was admitted with clinical features suggestive of restrictive cardiomyopathy, the underlying disorder was primary plasma cell leukemia. With chemotherapy the restrictive physiology was relieved supporting the diagnosis of plasma cell infiltration in the myocardium. We report this case due to rarity of the disease itself and its rare presentation.

Tuberculosis and vitamin D deficiency.

OBJECTIVES: a) To find out the normal level of 25 hydroxy vitamin D in healthy individuals b) To look for evidence of vitamin D deficiency in patients with active tuberculosis. METHODS: There were 35 cases of pulmonary and extra-pulmonary tuberculosis and 16 controls, whose clinical characteristics, dietary intake of vitamin D and biochemical characteristics including serum vitamin D levels were compared. EXCLUSION CRITERIA: malabsorption, liver or renal disorders, intake of drugs, which can reduce vitamin D levels, HIV infection, diabetes, immunosuppressive treatment, and severe protein energy malnutrition. RESULTS: There was a statistically significant difference (p < 0.005) in mean vitamin D levels between controls (19.5 ng/ml) and study subjects (10.7 ng/ml). Sixteen patients out of 35 had values well below the lower limit of normal (9 ng/ml). No one in the control group had vitamin D level less than 9 ng/ml. However the mean vitamin D level in the control group was less than the mean value quoted in the literature from the West. Sunlight exposure was adequate in those with deficiency but there was reduced dietary intake of vitamin D. CONCLUSIONS: Serum 25 hydroxy vitamin D levels less than 9 ng/ml indicates deficiency. Vitamin D deficiency exists in patients with tuberculosis and it is possibly a cause rather than effect of the disease; deficiency is due to decreased dietary intake. Vitamin D deficiency can occur without any symptoms. If symptoms are present, it indicates severe deficiency. Serum calcium and phosphorus values do not often predict the existence of deficiency.

Nail-patella syndrome with some unusual features.

Nail-patella syndrome is a rare hereditary disease. We report a patient who was the lone member affected in the family. The patient additionally had a bony defect of the skull which has not been reported so far in the literature.