Partha Pratim Chakraborty

Faulty Injection Technique: A Preventable But Often Overlooked Factor in Insulin Allergy

Insulin hypersensitivity—a rare occurrence with currently available insulin preparations—may have varied manifestations, ranging from a local injection site allergy to severe generalized anaphylactic reactions. While various additives included in commercial insulin preparations and insulin peptides themselves remain the primary allergens responsible, faulty injection technique may at times potentiate the development of insulin allergy. Management of insulin allergy is complex, potentially dangerous at times, and can be challenging for the treating physician. We report a case of insulin allergy due to intradermal insulin injections which was cured by adopting a proper injection technique.

Azathioprine-induced bullous Sweet's syndrome: a rare association

A 52-year-old man presented with high-grade fever, headache and painful vesicular skin rash involving the upper trunk and upper extremities, 8u2005days after initiation of chemotherapy with azathioprine (50u2005mg/day), which had been prescribed for acral vitiligo. There was neither any history of preceding respiratory or gastrointestinal tract infection, nor was the patient known to have malignancy, drug hypersensitivity, inflammatory bowel disease, vasculitis or other autoimmune disease. Laboratory results revealed leucocytosis with neutrophilia and markedly elevated acute phase reactants. Antinuclear antibody, perinuclear and cytoplasmic antineutrophil cytoplasmic antibody were found negative. Punch biopsy from skin of the upper trunk revealed dense neutrophilic infiltration of dermis without signs of vasculitis, suggestive of Sweets syndrome. In view of the temporal association with azathioprine and absence of an obvious alternative aetiology, provisional diagnosis of drug-induced bullous Sweets syndrome was made. Azathioprine was discontinued and high-dose oral prednisolone initiated. The response was dramatic with resolution of skin lesions within 72u2005h without further recurrence at fourth week of follow-up.

Consumptive hypothyroidism in solitary cutaneous haemangioma

A 2½-year-old boy had been diagnosed with primary hypothyroidism and was referred for persistently elevated thyroid stimulating hormone (TSH) despite regular supervised administration of a high dose of L-thyroxinexa0(15u2009µg/kg/day) for preceding 3u2009months. His developmental milestones (sitting with support at 6 months of age) were normal initially. His parents then noticed progressive lethargy, sleepiness, poor feeding, constipation, hoarse cry and delayed milestones that prompted a medical consultation. Thyroid function tests done a week after his birth for unconjugated hyperbilirubinaemia were normal. When the test was repeated at about 1.5 years of age, TSH came out to be 76 µIU/mL.nnClinical examination revealed short stature, facial puffiness, macroglossia with dry and coarse skin and a small umbilical hernia (figure 1). Thyroid was palpable at eutopic location. A cutaneous haemangioma (12xa0cm×10 cm) was present over the …

Distant site lipoatrophy: a rare complication of subcutaneous insulin therapy

Injection site lipodystrophy is not uncommon in insulin-treated patients with diabetes. In this era of human insulin and insulin analogues, lipoatrophy is far less common than lipohypertrophy, and distant site lipoatrophy is even rarer. The underlying pathophysiology of distant site lipoatrophy is largely undefined, and this disease is a diagnostic and therapeutic challenge to the treating physicians.nnA 60-year-old woman (BMI 32u2005kg/m2) with a 25-year history of type 2 diabetes mellitus presented with a depression in her interscapular region that had been present for 3u2005months and was causing a significant visible cosmetic defect (figure 1). Due to a diminished response to oral drugs, the patient had been prescribed a twice daily premixed human insulin regimen (30% regular …

Bilateral adrenal myelolipoma in Cushing's disease: a relook into the role of corticotropin in adrenal tumourigenesis

Adrenal myelolipomas are infrequently encountered benign tumours of unknown aetiology. In the majority of cases they are unilateral, and clinically and hormonally silent, only requiring periodic follow-up. However, bilateral adrenal myelolipomas are sometimes associated with endocrine disorders and warrant appropriate evaluation. Though the understanding of the pathophysiology of adrenal myelolipomas has long been elusive, adrenocorticotropic hormone (ACTH) has been proposed as the main tropic factor in a number of studies. Cushings disease is rarely associated with bilateral and sometimes giant myelolipomas. In this article, the association of bilateral adrenal myelolipomas with Cushings disease has been discussed and the role of ACTH in the tumourigenesis has been reviewed.

Pseudoacromegaly in pachydermoperiostosis.

A 19-year-old male patient presented with a year-long history of progressive painful enlargement of his hands and feet associated with excessive sweating and greasiness of the face, palms and soles. He also noticed pain over his wrists and ankles without any symptoms suggestive of raised intracranial tension and no recent increase in height. Family history was unremarkable. Clinical examination revealed enlargement of hands and feet with digital clubbing and spade-like appearance of the hands (figures 1 and 2). The eyelids were hypertrophied with partial drooping of the upper eyelids. The skin over the patients face and extremities was coarse, moist and oily, suggestive of excessive sebum secretion. He had no prognathism and no obvious furrowing of skin over face or scalp. Systemic examination was unremarkable.nnnnFigurexa01 nEnlarged spade-shaped hands with digital clubbing.nnnnnnFigurexa02 nEnlarged feet with swollen ankles.nnnnBaseline investigations were normal. A detailed hormonal evaluation including the growth hormone (GH) insulin-like growth factor 1 (IGF1) axis, …

Progressive pseudorheumatoid dysplasia: a close mimicker of juvenile idiopathic arthritis.

A girl aged 11½ years was referred to the endocrinology clinic with a provisional diagnosis of short stature secondary to juvenile idiopathic arthritis (JIA). Born of a consanguineous union, she had presented to her primary care physician with progressive stiffness and swelling of small joints of the hands and both ankles for the preceding 4u2005years. Her postnatal and early childhood periods were uneventful with normal developmental milestones.nnShe was short (height SD score (SDS) −3.8 and mid-parental height SDS −2.3) with an upper:lower segment ratio of 0.83, suggestive of short trunk dwarfism. Her breasts were of Tanner stage 3. The interphalangeal joints (both proximal and distal) of her hands (figure 1) and her ankles were swollen but non-tender on palpation. The axial skeleton and rest of the joints of the appendicular skeleton …

Split hand/foot malformation: a potential clue to underlying FGFR1 mutation in patients with isolated congenital hypogonadotropic hypogonadism

Congenital hypogonadotropic hypogonadismxa0(CHH) is characterised by absent or arrested pubertal development due to abnormal hypothalamic gonadotropin-releasing hormonexa0(GnRH) secretion or its action. A number of candidate genes (more than 20) have been identified to underlie CHH either acting alone or in combination. The prototype example of isolated CHH is Kallmann syndromexa0(KS) which has four subtypes: type 1xa0(mutation in anosmin gene), type 2xa0(mutation in fibroblast growth factor receptor 1 (FGFR1)), type 3xa0((mutation in prokinecitin receptor 2xa0(PROKR2)) and type 4xa0(mutation in prokinecitin). Loss-of-function mutations of FGFR1 or KS2 are responsible for about 10%–12% of patients with CHH. FGFR1 is expressed in a number of tissues, including the brain and skeletal system. Spontaneous reversal of CHH …

Insulin-induced thirst: are we overlooking it?

Time and again, we have been overwhelmed by little pearls of medicine which we gather along the way, in a journey to understand the functioning of human body in health and disease. In recent times, we came across two females with long-standing uncontrolled type 2 diabetes (HbA1c of more than 12%), who complained of an increased perception of thirst following sub-cutaneous insulin administration. They experienced thirst soon after initiating insulin that lasted for about 2–3 weeks. The response developed after about 30–60 min of each insulin shot. Capillary and plasma glucose values measured during thirst were between 120 and 150 mg/dl. Oblivious of the physiological effects of insulin on body water homeostasis, we had ignored the observation initially. The first lady even lost to follow-up as we disregarded her complaints repeatedly and attributed it to a psychiatric issue. The underlying mechanism of this dipsogenic response to insulin remains largely unknown. A study, done long back on a small group of human volunteers revealed significantly higher water intake after intravenous insulin injections when compared to saline. It was also observed that onset of water intake was concurrent with maximal dip in plasma glucose levels following intravenous insulin. In addition, plasma renin activity (PRA) and hematocrit levels were found to be significantly higher in the insulin-treated group when compared to basal conditions or after saline injections [1]. The effects of insulin-induced hypoglycemia on PRA were also assessed in subsets of normal human volunteers, adrenalectomised patients and patients with hypopituitarism. PRA, found to be significantly raised in all three groups, was amenable to blockade by propranolol [2]. A study assessing the role of glucose in insulin-induced drinking responses observed that the maximal dipsogenic response occurred during a rapid decline in blood glucose concentration which could be abolished by gastric administration of glucose. Thus, it appears that insulin-induced hypoglycemia results in a central sympathetic reflex stimulation of renin which leads to production of angiotensin II, a potent dipsogen. In all likelihood, this response is neither adrenal nor pituitary dependent, nor is the peripheral renin angiotensin system involved. Central angiotensin II, possibly, is the most important dipsogenic mediator underlying insulin-induced drinking response [3]. None of our patients had documented hypoglycemia during the thirst response. However, uncontrolled hyperglycemia for prolonged period is known to produce a right-ward shift of the set points of counter-regulatory hormone secretion and neuronal mechanisms of hypoglycemic response, a phenomenon known as relative hypoglycemia. Even more interesting is the fact that there seems to be a sex-specific difference in insulin-induced drinking, suggesting a role of gonadal steroids in neuronal structures or * Partha Pratim Chakraborty docparthapc@yahoo.co.in

Attenuated form of type II mucopolysaccharidoses (Hunter syndrome): pitfalls and potential clues in diagnosis

A 7-year-old boy, born of a non-consanguineous union, presented with pain over both hips and progressive difficulty in walking for preceding 2u2009years. He had normal developmental milestones with satisfactory scholastic performances and his family and perinatal histories were unremarkable. Clinical examination revealed short staturexa0(height SD score (SDS): −2.3; midparental heightxa0(MPH) SDS: −1.7) with upper segmentxa0(US):lower segmentxa0(LS) ratio of 0.97, umbilical hernia, apical pansystolic murmur of mitral regurgitation and waddling gait without corneal opacity or organomegalyxa0(figure 1). Complete blood count, baseline biochemistry and thyroid function tests were normal except presence of azurophilic granules within white blood cells seen in peripheral blood smearxa0(figure 2). Transthoracic echocardiography confirmed moderate mitral regurgitation secondary to prolapsed posterior mitral leaflet leading to coaptation failure. Though the difference between his height centile and MPH centile was not striking, his body proportion was suggestive of short trunk dwarfism (US:LS is normally 1.4 at 1u2009year and 1 at 10 years of age). A whole-body skeletal survey …