The patient, a 55 yr old female with Type 2 diabetes mellitus for the last 14 years, on insulin for 2 years, pre-sented with the complaint of sudden onset weakness of all four limbs for five days. She had a preceding history of severe muscle cramps and paraesthesia for about a month. History revealed that the patient had severe generalised weakness twenty days back, for which she had been admitted in a local hospital and had improved symptomatically (though some amount of muscle pain persisted) with intravenous fluid and injectable medications, the details of which could not be gathered. The onset of quadriparesis was acute, noticed on getting up in the morning, five days prior to admission in our institute. There was no history of unusually high carbohy-drate intake, cold exposure, excessive exertion or alcohol intake the previous night. She denied unexplained sweating, tremors, heat intolerance, prolonged vomiting, or any drug intake over a prolonged period of time. General examination revealed carpopedal spasm, hypertension(160/100 mm Hg) and mild pallor. Neurological examination revealed bilater-ally symmetrical weakness (Gr 1/5) of all four limbs and generalized hyporeflexia. Muscles of the eyes, face, tongue, pharynx, larynx, diaphragm and sphincters were not involved. Higher functions, cranial nerves and sensory system were intact. Plantar was bilaterally flexor. Apart from mild hepatomegaly, systemic examination was normal.\nInvestigations revealed hypokalemia(1.3 mEq/l), hypocalcemia (corrected 5.8 mg/dl) and hypomagnesemia (1.1 mg/dl) with normal sodium and chloride levels. Routine blood examination was normal. Fasting and post prandial plasma glucose was 171mg/dl and 309mg/dl respectively with an HBA1c of 9.5%. Serum osmolality was normal.\nUrinary ketones were negative. Liver function tests were slightly deranged with AST of 71 mg/dl and ALT of 186 mg/dl. Serum protein was 7.09mg/dl, albumin - 3.2 mg/dl, globulin - 3.8mg/dl, serum bilirubin -0.5mg/dl. Serum creatinine was 1.l mg/dl and microalbuminuria was present. CPK and LDH were within normal limits. Thyroid profile was normal. Serum phosphorus was normal and PTH was 50.3 pg/ml(normal–10 to 55 pg/ml). 24 hour urinary calcium was 61mg (normal - 100 to 300mg/24hrs). Urinary potas-sium excretion was 53 mEq/L /day (normal-25-120 mEq/L/day). Blood gas analysis revealed metabolic alkalosis. Electrocardiogram, ultrasound abdomen, plain picture abdomen, X-ray cervical spine and chest X-ray were normal. MRI of brain, spine and abdomen were also normal.\nInitially, the patient was treated with injectable potas-sium, magnesium and calcium preparations. Normalization of serum potassium and calcium was achieved only after total correction of magnesium level. There was gradual improve-ment in muscle power after 24 hours of therapy. Complete recovery was achieved after five days of treatment, following which she was shifted to oral preparations. Glycemic control was achieved using insulin and hypertension was controlled with Amlodepin 5 mg. She was discharged after two weeks on oral calcium(l gm daily) and advised to take diet rich in potassium and magnesium. On subsequent follow up at one and three months, she was asymptomatic with normal electrolyte levels and good glycemic control.
We present the case of a 15-year-old female heterozygous for ΔF508del/N1303K, with poorly controlled lung disease determined by forced expiratory volume in one second (FEV1) 44% of predicted value, who presented with cutaneous vasculitis and arthritis in association with an infective exacerbation of CF. The cutaneous involvement consisted of a palpable purpuric rash distributed over the tibial surfaces, ankles, and dorsa of the feet (Figure ). At this admission in 2015, infection and colonization with BCC and methicillin-resistant Staphylococcus aureus (MRSA) later in the year were also diagnosed. Blood work at admission is presented in Table ; acute phase reactants (C-reactive protein and fibrinogen) and rheumatoid factor were elevated. ANCAs were negative. Antinuclear antibodies (ANAs) were negative initially, but became positive five years later. Over the next several years, the patient required on average three to four hospitalizations every year due to IECF. Cutaneous vasculitis was present in approximately every other episode of IECF and improved with treatment of the pulmonary exacerbations. Bacterial colonization together with respiratory function is summarized in Figure - of note, the FEV1 decreased from 65% at 14 years of age (before appearance of the vasculitis) to 24% by 19 years of age.\nFrom the patient’s medical history, we consider eloquent the following facts: at the age of two years, she received nine-month isoniazid prophylaxis due to contact with her father who had tuberculosis. At the age of three years, she was diagnosed with bronchiectasis via CT. At the age of nine years, colonization with Pseudomonas aeruginosa was diagnosed through bronchoalveolar lavage and she had an FEV1 of 75% predicted. At the age of 13 years, the patient was diagnosed with a latent pulmonary tuberculosis infection which was again treated with nine months of isoniazid monotherapy. At the age of 14 years, she was diagnosed with allergic bronchopulmonary aspergillosis, via elevated total IgE and Aspergillus-specific IgE. At this time, the patient had an FEV1 of 65% predicted.\nAt the age of 15 years (2015), colonization with BCC was noted, she had an FEV1 of 44% predicted. At this time, the patient showed first signs of the cutaneous vasculitis, in form of a palpable purpuric, nonvesicular maculopapular rash localized to the lower legs. The lower extremities were slightly swollen, painful, and as the episodes of the vasculitis continued to erupt, grey discoloration in the place of the rash began to set in and over the years became permanent. At the age of 18 years (2018), colonization with Achromobacter xylosoxidans and Candida parapsilosis was noted, she had an FEV1 of 24% during the exacerbation, which improved to 38% of predicted after treatment. The chest x-ray comparison from 2015 to 2017 is presented in Figure and B, respectively. The CT scan done at this time showed cystic dilations in the right apex (Figure ) and severe bilateral middle lung field cylindrical bronchiectasis with signet-ring signs (Figure ).\nTreatment was started with long-term oral prednisone. Colonization with Pseudomonas, BCC, MRSA, Achromobacter xylosoxidans, and Candida parapsilosis was treated with multiple courses of inhaled, IV and oral antibiotics (linezolid, ceftazidime, ofloxacin) and antifungals (fluconazole and voriconasole). In addition, the following was administered: azithromycin (three days a week for three months), tiotropium, an antihistamine, and periodic cough assist with antibiotic aerosol treatment. Starting from 2019, the patient’s nebulization with colistin and tobramycin was supplemented with nightly positive-pressure ventilation (PPV), but the ventilation was discontinued six months later due to slight hemoptysis that was predominantly present in the morning. Throughout the six months, the patient was on PPV, she experienced twp IECF, which required hospitalization. The two episodes of IECF, together with the extensive bronchiectasis, may have contributed to the appearance of the hemoptysis, which may have predisposed certain areas of the lung to barotrauma. After the PPV was discontinued, the hemoptysis disappeared and did not require any more treatment.